Unusual glioma

52
Unusual Gliomas Dr vardan kulshreshtha M.cH Resident – final year Deptt. Of Neurosurgery RNT Medical College , Udaipur, INDIA 10/20/2016 1 unusual giomas

Transcript of Unusual glioma

Unusual Gliomas

Dr vardan kulshreshthaMcH Resident ndash final year

Deptt Of NeurosurgeryRNT Medical College Udaipur INDIA

10202016 1unusual giomas

bull These tumor deserve special considerationbull These are rare and there behaviour and

natural history is still largely less knownbull Many of these have indolent growth and

commonly reach to attention due to epilepsybull Routine use of IHC and genetic assay have led

to some understanding

10202016 2unusual giomas

Subependymal Giant Cell Astrocytomabull WHO grade 1bull Generally occur in

patients with tuberoussclerosis

bull Typical location isintraventricular

bull Present with features ofraised ICP andobstructive HCP

bull TUBEROUS Sclerosis isan autosomal dominantdisease

bull Mental retardationseizures adenomassebaceum

bull Altered skinpigmentations retinaltumors subungualfibromas tumors ofpancreas and spleen

10202016 3unusual giomas

Pathologybull Rarely undiagnosedbull Found typically in

intraventricular locationsbull Typical appearance of giant

cells mixed with cells ofastrocyte lineage

bull Well demarcated tumorswith modest infilteartioninto surrounding whitematter

bull Giant cells with abundanteosinophilic cytoplasm andspindle shaped nuclei

bull Mitoses nuclear atypianecrosis endothelialproliferation withaggressive behaviour

bull Anaplastic transformationis rare

bull IHC marker show positivityfor GFAP AND S-100

10202016 4unusual giomas

Imaging

bull CT demonstratescalcification as well ascontrast enhancement

10202016 5unusual giomas

MRI

bull Isointense tohypointense on T1 massin the periventricularlocation

bull Hyperintense signal onT2 WI

bull Strongly enhancedsignals on post contrast

10202016 6unusual giomas

Management

bull Do not require interventionunless bolck the foramen ofmonroe

bull Primary treatment issurgical total resection

bull Subtotal resection may beadequate in case totalresection is hazardous

bull Tumor rarely becomesaggressive

bull Tarnscortical transcallosalapproach

bull If surgery not possible ordenied by pt thenmanagement of HCP byshunt

bull Role of chemotherapy hasshown promises

10202016 7unusual giomas

Everolimus for Subependymal Giant-Cell Astrocytomas in Tuberous SclerosisDarcy A Krueger MD PhD Marguerite M Care MD Katherine Holland MD PhD KarenAgricola FNP Cynthia Tudor PNP Prajakta Mangeshkar MS Kimberly A Wilson MSAnna Byars PhD Tarek Sahmoud MD PhD and David Neal Franz MDN Engl J Med 2010 3631801-181

bull Backgroundbull Neurosurgical resection is the standard treatment for subependymal giant-cell astrocytomas in patients with the tuberous

sclerosis complex An alternative may be the use of everolimus which inhibits the mammalian target of rapamycin a protein regulated by gene products involved in the tuberous sclerosis complex

bull Methodsbull Patients 3 years of age or older with serial growth of subependymal giant-cell astrocytomas were eligible for this open-label

study The primary efficacy end point was the change in volume of subependymal giant-cell astrocytomas between baseline and 6 months We gave everolimus orally at a dose of 30 mg per square meter of body-surface area to achieve a trough concentration of 5 to 15 ng per milliliter

bull Resultsbull We enrolled 28 patients Everolimus therapy was associated with a clinically meaningful reduction in volume of the

primary subependymal giant-cell astrocytoma as assessed on independent central review (Plt0001 for baseline vs 6 months) with a reduction of at least 30 in 21 patients (75) and at least 50 in 9 patients (32) Marked reductions were seen within 3 months and were sustained There were no new lesions worsening hydrocephalus evidence of increased intracranial pressure or necessity for surgical resection or other therapy for subependymal giant-cell astrocytoma Of the 16 patients for whom 24-hour video electroencephalography data were available seizure frequency for the 6-month study period (vs the previous 6-month period) decreased in 9 did not change in 6 and increased in 1 (median change minus1 seizure P=002) The mean (plusmnSD) score on the validated Quality-of-Life in Childhood Epilepsy questionnaire (on which scores can range from 0 to 100 with higher scores indicating a better quality of life) was improved at 3 months (634plusmn124) and 6 months (621plusmn142) over the baseline score (578plusmn140) Single cases of grade 3 treatment-related sinusitis pneumonia viral bronchitis tooth infection stomatitis and leukopenia were reported

bull Conclusionsbull Everolimus therapy was associated with marked reduction in the volume of subependymal giant-cell

astrocytomas and seizure frequency and may be a potential alternative to neurosurgical resection in some cases though long-term studies are needed

10202016 8unusual giomas

Angiocentric glioma

bull Described by WHO in2005

bull Initially included withastroblastoma andchoroid glioma

bull Mean age at diagnosis is17 years

bull Pts present with longhistory of seizures

bull Microscopically show themonomorphous bipolarcells associated withnormal vessels of thecortex and white matter

bull Tumor cells are spindleshaped

bull Mitoses are rarebull Positive for S-100 GFAP

vimentin

10202016 9unusual giomas

Imaging

bull CT images show it as ahypodense lesion at thecortex

bull MR shows a mass withstalk towards theventricular surfacemdashpathognomic

bull Hypointense to iso onT1WI

bull Hyperintense on T2WIbull Post contrast

enhancement is sparse

10202016 10unusual giomas

10202016 11unusual giomas

Management and outcome

bull Follow an indolent coursebull Rare progressionbull Surgical cure obtained by total resectionbull Aggressive forms are exceedingly rare

10202016 12unusual giomas

bull Perm J 2013 Winter17(1)e100-2 doi 107812TPP12-060

bull The treatment of angiocentric glioma case report and literature review

bull Alexandru D1 Haghighi B Muhonen MG

bull Abstract

bull Angiocentric glioma is a recently described tumor recognized since 2007

by the World Health Organization Classification of Tumours of the Central

Nervous System We present the only case of angiocentric glioma at our

institution in the last 15 years and review the literature in an attempt to

establish prognostic parameters Our search revealed only 27 cases of

angiocentric glioma in the literature The most common presenting

symptom of angiocentric glioma was seizures Gross total resection of

the lesion was curative without need for radiation or chemotherapy

10202016 13unusual giomas

Astroblastoma

bull WHO grade 1bull Initially described by Bailey

and Bucy in 1930bull Has been a matter of debate

for similar features asanaplastic astrocytomaglioblastoma andgemistocytic astrocytomas

bull Commonly found in 1st

three decades of lifebull Female

preponderance(111)

bull Affects the hemispheresinvolving cortex subcortical andperiventricular regions

bull No infratentorail tumorhave been reported

bull Pts present with features ofraised ICP cortical deficitsseizures personalitychanges

10202016 14unusual giomas

bull Greatly variable naturalhistory

bull Have a slow andindolent course

bull Rarely show anaggressive outcome

bull Almost always definedby their grossappearance

bull Cut surface revealhomogenous soft pinkgrey substances

bull Cystic areas arefrequently encountered

bull The clusters of cellsform pseudorossette

10202016 15unusual giomas

bull Richly supplied byblood vessels

bull Nuclei of tumor cellsare away from bloodvessels and send fineprocesse to the vesselwall

bull High number of mitoticfigures can be found

bull Cellular atypia isfrequently noted

bull On IHC tumor cellsstain positive for GFAP

bull Positivity for vimentinNSE S-1OO EMA

bull These are positive foranti leu 7 antibodiesspecific for cells ofepithelial origin

bull Central necrosis can befound

10202016 16unusual giomas

Imaging

bull On CT the appearnacesvaries from poorlydefined hypodensetumor to welldemarcated and highlyenhancing masses

10202016 17unusual giomas

bull On MR image tumorsare hypointense towhite matter on T1

bull Hyperintense on T2bull Hyperintense on FLAIRbull Enhancement is seen

around the cystic centre

10202016 18unusual giomas

Management And Prognosis

bull Surgical resection is main treatmentbull Gross total resection can be easily achieved as

lesions are mostly in the cortical areasbull Therapeutic value is of radiation and

chemotherapy is uncertain as these are raretumors

bull Prediction of outcome is difficult as these remainindolent for a long time and may rapidlytransform to glioblastoma resulting in fataloutcomes

10202016 19unusual giomas

Pilomyxoid astrocytoma

bull Recently described tumorsbull Similarities with pilocytic

astrocytomasbull Until recently these were

grouped with PAbull These involve entire

neuraxisbull Mean age at preentation is

18 monthsbull Most are found in

hypothalamic ndash chiasmicregion

bull Pts present with features ofraised ICP

bull RAISED HEADCIRCUMFERENCE may beonly subtle sign in infants

bull Histologically consists ofmyxoid matrix with cells inloose fibrillary and myxoidbackground

bull Tumor cells formperivascular rosettes

10202016 20unusual giomas

bull Preferred sites includebull (1) optic nerve (lsquooptic

nerve gliomarsquo)bull (2) optic

chiasmhypothalamusbull (3) thalamus and basal

gangliabull (4) cerebral hemispherebull (5) cerebellum (lsquocerebellar

astrocytomarsquo) andbull (6) brainstem (dorsal

exophytic brainstemglioma)

bull Genetically chracterized bythe presence of NF1mutations BRAFduplications and theabsence of IDH1 mutation

10202016 21unusual giomas

Imaging

bull The huge chiasmatic-hypothalamic tumor revealslow signal intensity on aT1-weighted image (A)

bull Bright signal intensity on aT2-weighted image (B)

bull Gadolinium-enhancedbrain MR images showheterogeneously strongenhancement (C and D)

bull Leptomeningealdisseminations at thecervical spine levels

10202016 22unusual giomas

bull NO standard careprotocols have beendefined

bull As these are newlydefined and raretumors

bull Surgical resection isfavoured if locationfavours

bull Mean progression freesurvival is 25 months

bull Mean overall survival is60 months

bull Reduced survival isbecause of inability toachieve gross resection

10202016 23unusual giomas

bull The usual indications foradjuvant treatment(chemotherapy orradiation therapy)include tumor recurrenceafter initial completeresection or symptomatictumors

bull In addition adjuvanttreatment is generallyindicated for tumors withgrowth on follow-upimaging even in theabsence of symptoms

bull The role of chemotherapyis in evolution

bull Chemotherapy may beimplemented for the treatment ofinoperable or partially resectedgliomas

bull The decision to use postoperativeadjuvant therapy also depends ona lesions perceived malignanttendencies as the potentialbenefits of radiation therapy andchemotherapy must be weighedagainst their morbidity

bull The decision to use postoperativeadjuvant therapy also depends ona lesions perceived malignanttendencies as the potentialbenefits of radiation therapy andchemotherapy must be weighedagainst their morbidity

10202016 24unusual giomas

Pleomorphic xanthoastrocytoma

bull First described by Kepsand colleuges in 1979

bull Represents lt1 of allastrocytomas

bull May be found alongentire neuraxis mostlysupratentorial( temporallobe)

bull Cerebellum and soinalcord are the other sites

bull Pts present in 2nd and 3rd

decadesbull Median age at 14 yearsbull Both sexes are affected

equallybull 70-80 pts present with

seizures and headachefocal neuro deficits

bull Rarely tumor mayhemmorrhage

10202016 25unusual giomas

bull Supratentorail are mostoften cortical and meningesbased

bull Chronic seizuresbull Grossely these are firm

tumors variable in colorrelatively avascular

bull Typically invade the piaarachnoid space

bull 13 pts have involvementof all the three meninges

bull Cyst fluid is xanthochromic

bull Very specific microscpoiccriteria for diagnosis are

1 Pleomorphic andxanthomatous astrocytes

2 Perivascular lymphocytes3 Non infilterating histology4 Reticulin stainingbull Pleomorphism can be as

vast as glioblastoma orsarcomatous tumors

10202016 26unusual giomas

bull IHC Staining revealsspindle cells and roundpleomorphic cellspositive for S-100 andGFAP

bull Staining forsynaptophysin and NSEmay be also present

bull Some workers havereported them to be asubtype of ganglioglioma

bull Association between theNeurofibromatosis Sturge Weber Syndrome

bull Loss of chr 9 and gain ofchromosome 7 have beenfound

10202016 27unusual giomas

Imaging

bull CTbull PXAs are typically hypo

or isodense and may bewell or poorlydemarcated usually withno or little surroundingoedema

bull Calcification is rarebull Due to its superficial

location it may causescalloping of theoverlying bone

10202016 28unusual giomas

bull MRIbull T1

ndash solid component iso tohypointense cf grey matter

ndash cystic component low signalndash leptomeningeal involvement

seen in over 70 of casesbull T1 C+ (Gd)

ndash solid component usuallyenhances vividly

bull T2ndash solid component iso to

hyperintense cf grey matterndash cystic component high signalndash on T2 FLAIR sequence cystic

areas show hyperintensityrelative to CSF due to higherprotein contents

ndash little surrounding vasogenicoedema

10202016 29unusual giomas

Management amp Outcome

bull Quite rare tumorsbull Surgical excision appears to

be best favoured modalitybull Actuarial survival trates are

91 at 5 years and 82 at10 years

bull Median survival 18 years

bull Total resection should beattempted in every case asfar as possible

bull Resection of cyst lining isnot indicated

bull Data regarding the use ofadjuvant therapy at thistime are limited to few casereports only

bull Pts who had incompleteresection high mitoticindex necrosis should bereserved for strict vigilanceand CEMRI on follow ups

10202016 30unusual giomas

bull At progression repeatresection be attempted

bull Proression should betaken as aggressivetumor type

bull Malignanttransformation maynecessitate adjuvanttherapy

10202016 31unusual giomas

bull Br J Neurosurg 2013 Dec27(6)759-64 doi 103109026886972013776666 Epub 2013 Mar 20

bull Pleomorphic xanthoastrocytoma long-term results of surgical treatment and analysis of prognosticfactors

bull Gallo P1 Cecchi PC Locatelli F Rizzo P Ghimenton C Gerosa M Pinna G

bull BACKGROUND

bull Pleomorphic Xanthoastrocytoma (PXA) is a rare brain tumour most commonly affecting children andyoung adults To date only few data regarding the long-term follow-up of these patients after surgery are

available The aim of this study is to describe our single-institution experience in the surgicalmanagement of this particular glioma over a period of over 18 years

bull METHODS

bull We performed a retrospective review of all cases of PXA (40 patients) operated upon at theDepartment of Neurosurgery of Verona Italy between 1990 and 2008 The impact of clinical radiologicalsurgical and histological factors on overall survival (OS) and progression-free survival (PFS) was analysed bymeans of univariate and multivariate models

bull FINDINGS

bull We achieved a gross total resection (GTR) in 65 of patients Histological diagnosis was of grade II in 80anaplastic features were present in the remaining 20 Adjuvant treatment radiotherapy or chemo-

radiotherapy was administered in 40 of the cases Median follow-up was 74 months OS at 5- and10 years was 7632 and 6824 respectively PFS at 5- and 10 years was 71 and 58respectively In the multivariate model histological grade extent of resection and age at diagnosis (le 30years vs gt 30 years) were the only independent prognostic factors for both OS and PFS

bull CONCLUSIONS

bull Our retrospective long-term study confirms the relatively favourable prognosis associatedwith PXA Young patients with a low-grade tumour (WHO grade II) who underwent GTR carrythe longest OS and PFS

10202016 32unusual giomas

bull Brain Tumor Pathol 2006 Apr23(1)55-63

bull A case of anaplastic pleomorphic xanthoastrocytoma presenting with tumor bleeding and cerebrospinal fluid dissemination

bull Asano K1 Miyamoto S Kubo O Kikkukawa T Yagihashi A Ohkuma H

bull Pleomorphic xanthoastrocytoma (PXA) has been considered an astrocytic tumorwith a relatively favorable prognosis However PXA cases having several recurrent patterns with poor prognosis have been reported in recent years and a new concept of anaplastic PXA has been proposed The present case was a 59-year-old woman who presented with tumor bleeding onset and cerebrospinal fluid dissemination The patient had sudden-onset right hemiparesis aphasia and consciousness disturbance and was admitted to a local area hospital After emergency surgery had removed the hematoma postoperative contrast-enhanced CT scan revealed a left temporal tumor A second surgery was therefore performed for initial tumor removal 2 months later Histopathological findings showed that the tumor was typical PXA with strong pleomorphism and xanthomatous changes and contained an ependymoma-like component in the center area However endothelial proliferation and mitosis were more remarkable compared to ordinary PXA The MIB-1 labeling index was 98 high From these findings the histopathological diagnosis was anaplastic PXA The patient underwent surgery to remove recurrent tumors 5 and 16 months later The patient died 36 months after the first onset and CT revealed glioblastoma-like

findings and cerebrospinal fluid dissemination This case report is the first case in which PXA presented with tumor bleeding onset Histopathological findings suggested anaplastic PXA from the first surgical specimens and PXA recurred many times We thus believe that the patient displayed primary anaplastic PXA rather than secondary anaplastic PXA that results in malignant transformation10202016 33unusual giomas

Ganglioglioma

bull WHO grade 1bull Initially described by

Perkins in 1926bull Benign well

differentiatedneuroepithelial tumors

bull Most commonly found intemporal lobe though canoccur anywhere in CNS

bull Commonly found inyoung patients withepilepsy

bull Most commonly notedtumor in temporal lobeepilepsy patients

bull Have been described inbrain steem spinal cordand cerebellum

bull Account for 1 ofprimary brain tumors

10202016 34unusual giomas

bull Male dominencebull Most common features

are seizures headachedizziness ataxiaprogressive weakness

bull Malignanttransformation is rare

bull Histology showsimmature mixture ofabnormal neural andglial elements

bull Macroscopically appearsolid or cystic

bull Cystic are welldelineatedfromsurrounding brain

bull Calcification is common

10202016 35unusual giomas

bull IHC staing shows reactivity for GFAP S-100 andvimentin

bull Neurons show synaptophysin class III betatubulin NFP chromogranin reactivity

10202016 36unusual giomas

Imaging

bull Highly variable imagingcharacters

bull No special typicalimaging criteria

bull Enhancement patternsare highly variable

bull Iso to hypointense onT1WI

bull Heterogenous andhyperintense on T2WI

bull Can contain solid cyticcalcified components

10202016 37unusual giomas

bull FDG PET scan showshypometabolism oreumetabolism in thesepatients

10202016 38unusual giomas

Management And Outcome

bull Gold standard is grosstotal resection

bull Seizure control aftersurgery is as high as80

bull Survival rates are highbull Event free survival of

low grade and highgrade gangiogioma are57 vs 15

bull The optimal surgicalresection depends uponthe eloquent areasinvolved in the vicinityof the tumor

bull The use ofchemotherapy is notwell established

bull Typically they shouldbe allowed for chemoand radiotherapy

10202016 39unusual giomas

bull The use of adjuvant therapy is warranted in thosecases which harbour residual lesions or features ofanaplasia

bull Potential harm to nearby brain areas preclude thejudicious use of radiation

10202016 40unusual giomas

bull Brain Tumor Res Treat 2014 Oct 2(2) 49ndash55

bull Treatment and Outcomes for Gangliogliomas A Single-Center Review of 16 Patients

bull Jye Young Song Jeong Hoon Kim Young Hyun Cho Chang Jin Kim and Eun Jung Lee

bull Ganglioglioma is a rare and slowly growing benign tumor We investigated the outcomes of patients who underwent different combination treatments

bull Methods

bull Between 1998 and 2012 16 patients including 11 men and 5 women with a median age of 125 years (range 25-65 years) were treated for intracranial gangliogliomas at our institution The median follow-up period was 57 years (range 48 days-156 years) Fifteen cases were included in the outcome assessment because one patient was lost to follow-up Complete resection was achieved in 8 (53) patients Six (40) patients underwent incomplete resection with or without adjuvant radiotherapy and one patient with a brainstem tumor underwent only stereotactic biopsy

bull Results

bull Gangliogliomas predominantly affected young (875) male patients and most frequently presented with seizures (64) Of eight patients who underwent complete resection seven did not show recurrence whereas only three of six with incomplete resection showed no recurrence Four patients with recurrence received salvage treatments (two repeat surgeries and two radiosurgeries) A tumor control rate of 93 (1415) was achieved at the last follow-up No recurrence or malignant changes were observed after a median follow-up of 12 and 45 years in four patients who received gamma knife (GK) radiosurgery as adjuvant and salvage treatment

bull Conclusion

bull Complete resection produced the best outcomes and incomplete resection followed by adjuvant or salvage treatments showed favorable outcomes In patients who are not eligible for complete resection because of tumor location or potential neurologic deficits following surgery GK radiosurgery should be considered for the treatment of residual or recurrent tumors

10202016 41unusual giomas

Papillary tumours of pineal

bull WHO grade II AND IIIbull Relatively new edition to WHObull They are equal in both sexesbull Reach medical attention due to headache mental

status changes and altered visionbull Contain eosinophilic cells with distinctive

borders with large pleomorphic nucleibull Positive for cytokeratin synaptophysin stain

variably for GFAP vimentin S-100

10202016 unusual giomas 42

Imaging

bull Generally found to be diffusely enhancinglesion of pineal region

bull Post 3rd ventricle with HCPbull Appear well demarcated from thalamus and

cerebellum

10202016 unusual giomas 43

Mangement

bull Standard treatment is excisionbull Grow slowly and donot transform to

malignant tumorsbull Local recurrence is however possiblebull Radiotherapy appears to provide good control

in local recurrence

10202016 unusual giomas 44

Choroid Glioma Of Third Ventricle

bull WHO Grade IIbull Ist recognised by WHO in

1998bull Arise in the anterior third

ventriclebull Commonly come to

attention after HCPbull Appera to contain in 3rd

ventricle and thought toarise from ventricularsurface

bull Composed of GFAP andvimentin positive cells inmucinous matrix

bull Nuclei are uniform insize and shape

bull Indolent growthbull May show EGF receptor

positivitybull Some suggets these tumor

arise from the lminaterminalis andventricular tanycytes

10202016 45unusual giomas

Imaging

bull Commonly fill 3rd

ventriclebull Diffusely enhance with

contrastbull Appear to infiltrate the

thalamusbull Large tumors may be

difficult to differentiatefrom suprasellar masses

10202016 46unusual giomas

10202016 47unusual giomas

bull Surgical management is limited by locationbull Access through the dialted ventricle and lamina

terminalis may be challangingbull Subtotal resection is thus commonbull Role of adjuvant radiotherapy is unknown

10202016 48unusual giomas

bull Acta Neurochir (Wien) 2008 Nov150(11)1183-91 doi 101007s00701-008-0014-6 Epub

2008 Oct 21

bull Chordoid glioma of the third ventricle

bull Vanhauwaert DJ1 Clement F Van Dorpe J Deruytter MJKGROUND

bull Chordoid glioma is a rare tumour (World Health Organisation grade II) originating from the third ventricle

with both glial and chordoid features It was first described by Brat in 1998 Since there is no detailed

information available on the outcome after surgery and adjuvant treatment we reviewed the literature

bull METHODS

bull A literature search through PUBMED revealed 50 cases of chordoid glioma Most reports

were found in pathology journals Information on the postoperative course was sometimes

very limited We reviewed the available literature and studied in detail the presenting

symptoms mortality and postoperative complications in relation to the extent of resective

surgery as well as the importance of adjuvant treatment

bull CONCLUSIONS

bull Mortality in the immediate postoperative period is 32 and is higher after gross total

resection as compared to subtotal resection Non-fatal postoperative complications are

hypothalamic disorders and mental alterations Gross total resection is the treatment of

choice since no recurrence has been reported after macroscopically complete resection

but this is often difficult because of the location and adherence to the hypothalamus The

role of postoperative radiotherapy is uncertain There is some indication that radiosurgery

with or without conventional irradiation is superior to conventional radiation alone Planned

subtotal resection followed by stereotactic radiosurgery can be a safe and effective

alternative in a patient in whom gross total resection is considered to be too risky

There is no report on the use of chemotherapy in the treatment of chordoid gliomas More

information about the optimal treatment strategy is needed and more reports are also needed10202016 49unusual giomas

bull Neurologist 2011 Jan17(1)52bull Chordoid glioma a case report and literature reviewbull Liu WP1 Cheng JX Yi XC Zhen HN Fei Z Li Q Zhang Xbull chordoid glioma is a rare tumor (World Health Organization grade II) with both

glial and chordoid features often located in the suprasellar region and anterior third ventricle It was first described by Brat in 1998 Because there is no detailed information available from the clinical perspective we reviewed the literature

bull METHODS

bull a literature search through PUBMED and CNKI revealed 64 cases of chordoid glioma Information on the clinical course was very limited We reviewed the literature and studied the pathologic and imaging features postoperative mortality and morbidity in relation to surgical extension and approaches and the importance of adjuvant treatment

bull CONCLUSIONS bull mortality in the immediate postoperative period is 28 and postoperative

morbidity is 60 which are statistically higher after gross total resection as compared with subtotal resection Translamina terminalis approach is considered to be the best approach The current study cannot document that patients have longer survival and higher quality of life after gross total resection than subtotal resection The role of postoperative radiotherapy is uncertain and there is no report on the use of chemotherapy More information about the optimal treatment strategy is needed

10202016 50unusual giomas

Conclusion

bull They are identified by their unique morphologicalfeatures

bull Many of these have indolent course and identified byepilepsy

bull Potentail diagnosis should be considered in youngpatients with seizures and cortically based lesions

bull Correct diagnosis requires experiencedneuropathologisrt

bull As more experienced is gained more knowledgetowards better understanding shall provide betteroutcomes in these patients

10202016 unusual giomas 51

10202016 52unusual giomas

Content supportndash youmann neurosurgery textbook

bull These tumor deserve special considerationbull These are rare and there behaviour and

natural history is still largely less knownbull Many of these have indolent growth and

commonly reach to attention due to epilepsybull Routine use of IHC and genetic assay have led

to some understanding

10202016 2unusual giomas

Subependymal Giant Cell Astrocytomabull WHO grade 1bull Generally occur in

patients with tuberoussclerosis

bull Typical location isintraventricular

bull Present with features ofraised ICP andobstructive HCP

bull TUBEROUS Sclerosis isan autosomal dominantdisease

bull Mental retardationseizures adenomassebaceum

bull Altered skinpigmentations retinaltumors subungualfibromas tumors ofpancreas and spleen

10202016 3unusual giomas

Pathologybull Rarely undiagnosedbull Found typically in

intraventricular locationsbull Typical appearance of giant

cells mixed with cells ofastrocyte lineage

bull Well demarcated tumorswith modest infilteartioninto surrounding whitematter

bull Giant cells with abundanteosinophilic cytoplasm andspindle shaped nuclei

bull Mitoses nuclear atypianecrosis endothelialproliferation withaggressive behaviour

bull Anaplastic transformationis rare

bull IHC marker show positivityfor GFAP AND S-100

10202016 4unusual giomas

Imaging

bull CT demonstratescalcification as well ascontrast enhancement

10202016 5unusual giomas

MRI

bull Isointense tohypointense on T1 massin the periventricularlocation

bull Hyperintense signal onT2 WI

bull Strongly enhancedsignals on post contrast

10202016 6unusual giomas

Management

bull Do not require interventionunless bolck the foramen ofmonroe

bull Primary treatment issurgical total resection

bull Subtotal resection may beadequate in case totalresection is hazardous

bull Tumor rarely becomesaggressive

bull Tarnscortical transcallosalapproach

bull If surgery not possible ordenied by pt thenmanagement of HCP byshunt

bull Role of chemotherapy hasshown promises

10202016 7unusual giomas

Everolimus for Subependymal Giant-Cell Astrocytomas in Tuberous SclerosisDarcy A Krueger MD PhD Marguerite M Care MD Katherine Holland MD PhD KarenAgricola FNP Cynthia Tudor PNP Prajakta Mangeshkar MS Kimberly A Wilson MSAnna Byars PhD Tarek Sahmoud MD PhD and David Neal Franz MDN Engl J Med 2010 3631801-181

bull Backgroundbull Neurosurgical resection is the standard treatment for subependymal giant-cell astrocytomas in patients with the tuberous

sclerosis complex An alternative may be the use of everolimus which inhibits the mammalian target of rapamycin a protein regulated by gene products involved in the tuberous sclerosis complex

bull Methodsbull Patients 3 years of age or older with serial growth of subependymal giant-cell astrocytomas were eligible for this open-label

study The primary efficacy end point was the change in volume of subependymal giant-cell astrocytomas between baseline and 6 months We gave everolimus orally at a dose of 30 mg per square meter of body-surface area to achieve a trough concentration of 5 to 15 ng per milliliter

bull Resultsbull We enrolled 28 patients Everolimus therapy was associated with a clinically meaningful reduction in volume of the

primary subependymal giant-cell astrocytoma as assessed on independent central review (Plt0001 for baseline vs 6 months) with a reduction of at least 30 in 21 patients (75) and at least 50 in 9 patients (32) Marked reductions were seen within 3 months and were sustained There were no new lesions worsening hydrocephalus evidence of increased intracranial pressure or necessity for surgical resection or other therapy for subependymal giant-cell astrocytoma Of the 16 patients for whom 24-hour video electroencephalography data were available seizure frequency for the 6-month study period (vs the previous 6-month period) decreased in 9 did not change in 6 and increased in 1 (median change minus1 seizure P=002) The mean (plusmnSD) score on the validated Quality-of-Life in Childhood Epilepsy questionnaire (on which scores can range from 0 to 100 with higher scores indicating a better quality of life) was improved at 3 months (634plusmn124) and 6 months (621plusmn142) over the baseline score (578plusmn140) Single cases of grade 3 treatment-related sinusitis pneumonia viral bronchitis tooth infection stomatitis and leukopenia were reported

bull Conclusionsbull Everolimus therapy was associated with marked reduction in the volume of subependymal giant-cell

astrocytomas and seizure frequency and may be a potential alternative to neurosurgical resection in some cases though long-term studies are needed

10202016 8unusual giomas

Angiocentric glioma

bull Described by WHO in2005

bull Initially included withastroblastoma andchoroid glioma

bull Mean age at diagnosis is17 years

bull Pts present with longhistory of seizures

bull Microscopically show themonomorphous bipolarcells associated withnormal vessels of thecortex and white matter

bull Tumor cells are spindleshaped

bull Mitoses are rarebull Positive for S-100 GFAP

vimentin

10202016 9unusual giomas

Imaging

bull CT images show it as ahypodense lesion at thecortex

bull MR shows a mass withstalk towards theventricular surfacemdashpathognomic

bull Hypointense to iso onT1WI

bull Hyperintense on T2WIbull Post contrast

enhancement is sparse

10202016 10unusual giomas

10202016 11unusual giomas

Management and outcome

bull Follow an indolent coursebull Rare progressionbull Surgical cure obtained by total resectionbull Aggressive forms are exceedingly rare

10202016 12unusual giomas

bull Perm J 2013 Winter17(1)e100-2 doi 107812TPP12-060

bull The treatment of angiocentric glioma case report and literature review

bull Alexandru D1 Haghighi B Muhonen MG

bull Abstract

bull Angiocentric glioma is a recently described tumor recognized since 2007

by the World Health Organization Classification of Tumours of the Central

Nervous System We present the only case of angiocentric glioma at our

institution in the last 15 years and review the literature in an attempt to

establish prognostic parameters Our search revealed only 27 cases of

angiocentric glioma in the literature The most common presenting

symptom of angiocentric glioma was seizures Gross total resection of

the lesion was curative without need for radiation or chemotherapy

10202016 13unusual giomas

Astroblastoma

bull WHO grade 1bull Initially described by Bailey

and Bucy in 1930bull Has been a matter of debate

for similar features asanaplastic astrocytomaglioblastoma andgemistocytic astrocytomas

bull Commonly found in 1st

three decades of lifebull Female

preponderance(111)

bull Affects the hemispheresinvolving cortex subcortical andperiventricular regions

bull No infratentorail tumorhave been reported

bull Pts present with features ofraised ICP cortical deficitsseizures personalitychanges

10202016 14unusual giomas

bull Greatly variable naturalhistory

bull Have a slow andindolent course

bull Rarely show anaggressive outcome

bull Almost always definedby their grossappearance

bull Cut surface revealhomogenous soft pinkgrey substances

bull Cystic areas arefrequently encountered

bull The clusters of cellsform pseudorossette

10202016 15unusual giomas

bull Richly supplied byblood vessels

bull Nuclei of tumor cellsare away from bloodvessels and send fineprocesse to the vesselwall

bull High number of mitoticfigures can be found

bull Cellular atypia isfrequently noted

bull On IHC tumor cellsstain positive for GFAP

bull Positivity for vimentinNSE S-1OO EMA

bull These are positive foranti leu 7 antibodiesspecific for cells ofepithelial origin

bull Central necrosis can befound

10202016 16unusual giomas

Imaging

bull On CT the appearnacesvaries from poorlydefined hypodensetumor to welldemarcated and highlyenhancing masses

10202016 17unusual giomas

bull On MR image tumorsare hypointense towhite matter on T1

bull Hyperintense on T2bull Hyperintense on FLAIRbull Enhancement is seen

around the cystic centre

10202016 18unusual giomas

Management And Prognosis

bull Surgical resection is main treatmentbull Gross total resection can be easily achieved as

lesions are mostly in the cortical areasbull Therapeutic value is of radiation and

chemotherapy is uncertain as these are raretumors

bull Prediction of outcome is difficult as these remainindolent for a long time and may rapidlytransform to glioblastoma resulting in fataloutcomes

10202016 19unusual giomas

Pilomyxoid astrocytoma

bull Recently described tumorsbull Similarities with pilocytic

astrocytomasbull Until recently these were

grouped with PAbull These involve entire

neuraxisbull Mean age at preentation is

18 monthsbull Most are found in

hypothalamic ndash chiasmicregion

bull Pts present with features ofraised ICP

bull RAISED HEADCIRCUMFERENCE may beonly subtle sign in infants

bull Histologically consists ofmyxoid matrix with cells inloose fibrillary and myxoidbackground

bull Tumor cells formperivascular rosettes

10202016 20unusual giomas

bull Preferred sites includebull (1) optic nerve (lsquooptic

nerve gliomarsquo)bull (2) optic

chiasmhypothalamusbull (3) thalamus and basal

gangliabull (4) cerebral hemispherebull (5) cerebellum (lsquocerebellar

astrocytomarsquo) andbull (6) brainstem (dorsal

exophytic brainstemglioma)

bull Genetically chracterized bythe presence of NF1mutations BRAFduplications and theabsence of IDH1 mutation

10202016 21unusual giomas

Imaging

bull The huge chiasmatic-hypothalamic tumor revealslow signal intensity on aT1-weighted image (A)

bull Bright signal intensity on aT2-weighted image (B)

bull Gadolinium-enhancedbrain MR images showheterogeneously strongenhancement (C and D)

bull Leptomeningealdisseminations at thecervical spine levels

10202016 22unusual giomas

bull NO standard careprotocols have beendefined

bull As these are newlydefined and raretumors

bull Surgical resection isfavoured if locationfavours

bull Mean progression freesurvival is 25 months

bull Mean overall survival is60 months

bull Reduced survival isbecause of inability toachieve gross resection

10202016 23unusual giomas

bull The usual indications foradjuvant treatment(chemotherapy orradiation therapy)include tumor recurrenceafter initial completeresection or symptomatictumors

bull In addition adjuvanttreatment is generallyindicated for tumors withgrowth on follow-upimaging even in theabsence of symptoms

bull The role of chemotherapyis in evolution

bull Chemotherapy may beimplemented for the treatment ofinoperable or partially resectedgliomas

bull The decision to use postoperativeadjuvant therapy also depends ona lesions perceived malignanttendencies as the potentialbenefits of radiation therapy andchemotherapy must be weighedagainst their morbidity

bull The decision to use postoperativeadjuvant therapy also depends ona lesions perceived malignanttendencies as the potentialbenefits of radiation therapy andchemotherapy must be weighedagainst their morbidity

10202016 24unusual giomas

Pleomorphic xanthoastrocytoma

bull First described by Kepsand colleuges in 1979

bull Represents lt1 of allastrocytomas

bull May be found alongentire neuraxis mostlysupratentorial( temporallobe)

bull Cerebellum and soinalcord are the other sites

bull Pts present in 2nd and 3rd

decadesbull Median age at 14 yearsbull Both sexes are affected

equallybull 70-80 pts present with

seizures and headachefocal neuro deficits

bull Rarely tumor mayhemmorrhage

10202016 25unusual giomas

bull Supratentorail are mostoften cortical and meningesbased

bull Chronic seizuresbull Grossely these are firm

tumors variable in colorrelatively avascular

bull Typically invade the piaarachnoid space

bull 13 pts have involvementof all the three meninges

bull Cyst fluid is xanthochromic

bull Very specific microscpoiccriteria for diagnosis are

1 Pleomorphic andxanthomatous astrocytes

2 Perivascular lymphocytes3 Non infilterating histology4 Reticulin stainingbull Pleomorphism can be as

vast as glioblastoma orsarcomatous tumors

10202016 26unusual giomas

bull IHC Staining revealsspindle cells and roundpleomorphic cellspositive for S-100 andGFAP

bull Staining forsynaptophysin and NSEmay be also present

bull Some workers havereported them to be asubtype of ganglioglioma

bull Association between theNeurofibromatosis Sturge Weber Syndrome

bull Loss of chr 9 and gain ofchromosome 7 have beenfound

10202016 27unusual giomas

Imaging

bull CTbull PXAs are typically hypo

or isodense and may bewell or poorlydemarcated usually withno or little surroundingoedema

bull Calcification is rarebull Due to its superficial

location it may causescalloping of theoverlying bone

10202016 28unusual giomas

bull MRIbull T1

ndash solid component iso tohypointense cf grey matter

ndash cystic component low signalndash leptomeningeal involvement

seen in over 70 of casesbull T1 C+ (Gd)

ndash solid component usuallyenhances vividly

bull T2ndash solid component iso to

hyperintense cf grey matterndash cystic component high signalndash on T2 FLAIR sequence cystic

areas show hyperintensityrelative to CSF due to higherprotein contents

ndash little surrounding vasogenicoedema

10202016 29unusual giomas

Management amp Outcome

bull Quite rare tumorsbull Surgical excision appears to

be best favoured modalitybull Actuarial survival trates are

91 at 5 years and 82 at10 years

bull Median survival 18 years

bull Total resection should beattempted in every case asfar as possible

bull Resection of cyst lining isnot indicated

bull Data regarding the use ofadjuvant therapy at thistime are limited to few casereports only

bull Pts who had incompleteresection high mitoticindex necrosis should bereserved for strict vigilanceand CEMRI on follow ups

10202016 30unusual giomas

bull At progression repeatresection be attempted

bull Proression should betaken as aggressivetumor type

bull Malignanttransformation maynecessitate adjuvanttherapy

10202016 31unusual giomas

bull Br J Neurosurg 2013 Dec27(6)759-64 doi 103109026886972013776666 Epub 2013 Mar 20

bull Pleomorphic xanthoastrocytoma long-term results of surgical treatment and analysis of prognosticfactors

bull Gallo P1 Cecchi PC Locatelli F Rizzo P Ghimenton C Gerosa M Pinna G

bull BACKGROUND

bull Pleomorphic Xanthoastrocytoma (PXA) is a rare brain tumour most commonly affecting children andyoung adults To date only few data regarding the long-term follow-up of these patients after surgery are

available The aim of this study is to describe our single-institution experience in the surgicalmanagement of this particular glioma over a period of over 18 years

bull METHODS

bull We performed a retrospective review of all cases of PXA (40 patients) operated upon at theDepartment of Neurosurgery of Verona Italy between 1990 and 2008 The impact of clinical radiologicalsurgical and histological factors on overall survival (OS) and progression-free survival (PFS) was analysed bymeans of univariate and multivariate models

bull FINDINGS

bull We achieved a gross total resection (GTR) in 65 of patients Histological diagnosis was of grade II in 80anaplastic features were present in the remaining 20 Adjuvant treatment radiotherapy or chemo-

radiotherapy was administered in 40 of the cases Median follow-up was 74 months OS at 5- and10 years was 7632 and 6824 respectively PFS at 5- and 10 years was 71 and 58respectively In the multivariate model histological grade extent of resection and age at diagnosis (le 30years vs gt 30 years) were the only independent prognostic factors for both OS and PFS

bull CONCLUSIONS

bull Our retrospective long-term study confirms the relatively favourable prognosis associatedwith PXA Young patients with a low-grade tumour (WHO grade II) who underwent GTR carrythe longest OS and PFS

10202016 32unusual giomas

bull Brain Tumor Pathol 2006 Apr23(1)55-63

bull A case of anaplastic pleomorphic xanthoastrocytoma presenting with tumor bleeding and cerebrospinal fluid dissemination

bull Asano K1 Miyamoto S Kubo O Kikkukawa T Yagihashi A Ohkuma H

bull Pleomorphic xanthoastrocytoma (PXA) has been considered an astrocytic tumorwith a relatively favorable prognosis However PXA cases having several recurrent patterns with poor prognosis have been reported in recent years and a new concept of anaplastic PXA has been proposed The present case was a 59-year-old woman who presented with tumor bleeding onset and cerebrospinal fluid dissemination The patient had sudden-onset right hemiparesis aphasia and consciousness disturbance and was admitted to a local area hospital After emergency surgery had removed the hematoma postoperative contrast-enhanced CT scan revealed a left temporal tumor A second surgery was therefore performed for initial tumor removal 2 months later Histopathological findings showed that the tumor was typical PXA with strong pleomorphism and xanthomatous changes and contained an ependymoma-like component in the center area However endothelial proliferation and mitosis were more remarkable compared to ordinary PXA The MIB-1 labeling index was 98 high From these findings the histopathological diagnosis was anaplastic PXA The patient underwent surgery to remove recurrent tumors 5 and 16 months later The patient died 36 months after the first onset and CT revealed glioblastoma-like

findings and cerebrospinal fluid dissemination This case report is the first case in which PXA presented with tumor bleeding onset Histopathological findings suggested anaplastic PXA from the first surgical specimens and PXA recurred many times We thus believe that the patient displayed primary anaplastic PXA rather than secondary anaplastic PXA that results in malignant transformation10202016 33unusual giomas

Ganglioglioma

bull WHO grade 1bull Initially described by

Perkins in 1926bull Benign well

differentiatedneuroepithelial tumors

bull Most commonly found intemporal lobe though canoccur anywhere in CNS

bull Commonly found inyoung patients withepilepsy

bull Most commonly notedtumor in temporal lobeepilepsy patients

bull Have been described inbrain steem spinal cordand cerebellum

bull Account for 1 ofprimary brain tumors

10202016 34unusual giomas

bull Male dominencebull Most common features

are seizures headachedizziness ataxiaprogressive weakness

bull Malignanttransformation is rare

bull Histology showsimmature mixture ofabnormal neural andglial elements

bull Macroscopically appearsolid or cystic

bull Cystic are welldelineatedfromsurrounding brain

bull Calcification is common

10202016 35unusual giomas

bull IHC staing shows reactivity for GFAP S-100 andvimentin

bull Neurons show synaptophysin class III betatubulin NFP chromogranin reactivity

10202016 36unusual giomas

Imaging

bull Highly variable imagingcharacters

bull No special typicalimaging criteria

bull Enhancement patternsare highly variable

bull Iso to hypointense onT1WI

bull Heterogenous andhyperintense on T2WI

bull Can contain solid cyticcalcified components

10202016 37unusual giomas

bull FDG PET scan showshypometabolism oreumetabolism in thesepatients

10202016 38unusual giomas

Management And Outcome

bull Gold standard is grosstotal resection

bull Seizure control aftersurgery is as high as80

bull Survival rates are highbull Event free survival of

low grade and highgrade gangiogioma are57 vs 15

bull The optimal surgicalresection depends uponthe eloquent areasinvolved in the vicinityof the tumor

bull The use ofchemotherapy is notwell established

bull Typically they shouldbe allowed for chemoand radiotherapy

10202016 39unusual giomas

bull The use of adjuvant therapy is warranted in thosecases which harbour residual lesions or features ofanaplasia

bull Potential harm to nearby brain areas preclude thejudicious use of radiation

10202016 40unusual giomas

bull Brain Tumor Res Treat 2014 Oct 2(2) 49ndash55

bull Treatment and Outcomes for Gangliogliomas A Single-Center Review of 16 Patients

bull Jye Young Song Jeong Hoon Kim Young Hyun Cho Chang Jin Kim and Eun Jung Lee

bull Ganglioglioma is a rare and slowly growing benign tumor We investigated the outcomes of patients who underwent different combination treatments

bull Methods

bull Between 1998 and 2012 16 patients including 11 men and 5 women with a median age of 125 years (range 25-65 years) were treated for intracranial gangliogliomas at our institution The median follow-up period was 57 years (range 48 days-156 years) Fifteen cases were included in the outcome assessment because one patient was lost to follow-up Complete resection was achieved in 8 (53) patients Six (40) patients underwent incomplete resection with or without adjuvant radiotherapy and one patient with a brainstem tumor underwent only stereotactic biopsy

bull Results

bull Gangliogliomas predominantly affected young (875) male patients and most frequently presented with seizures (64) Of eight patients who underwent complete resection seven did not show recurrence whereas only three of six with incomplete resection showed no recurrence Four patients with recurrence received salvage treatments (two repeat surgeries and two radiosurgeries) A tumor control rate of 93 (1415) was achieved at the last follow-up No recurrence or malignant changes were observed after a median follow-up of 12 and 45 years in four patients who received gamma knife (GK) radiosurgery as adjuvant and salvage treatment

bull Conclusion

bull Complete resection produced the best outcomes and incomplete resection followed by adjuvant or salvage treatments showed favorable outcomes In patients who are not eligible for complete resection because of tumor location or potential neurologic deficits following surgery GK radiosurgery should be considered for the treatment of residual or recurrent tumors

10202016 41unusual giomas

Papillary tumours of pineal

bull WHO grade II AND IIIbull Relatively new edition to WHObull They are equal in both sexesbull Reach medical attention due to headache mental

status changes and altered visionbull Contain eosinophilic cells with distinctive

borders with large pleomorphic nucleibull Positive for cytokeratin synaptophysin stain

variably for GFAP vimentin S-100

10202016 unusual giomas 42

Imaging

bull Generally found to be diffusely enhancinglesion of pineal region

bull Post 3rd ventricle with HCPbull Appear well demarcated from thalamus and

cerebellum

10202016 unusual giomas 43

Mangement

bull Standard treatment is excisionbull Grow slowly and donot transform to

malignant tumorsbull Local recurrence is however possiblebull Radiotherapy appears to provide good control

in local recurrence

10202016 unusual giomas 44

Choroid Glioma Of Third Ventricle

bull WHO Grade IIbull Ist recognised by WHO in

1998bull Arise in the anterior third

ventriclebull Commonly come to

attention after HCPbull Appera to contain in 3rd

ventricle and thought toarise from ventricularsurface

bull Composed of GFAP andvimentin positive cells inmucinous matrix

bull Nuclei are uniform insize and shape

bull Indolent growthbull May show EGF receptor

positivitybull Some suggets these tumor

arise from the lminaterminalis andventricular tanycytes

10202016 45unusual giomas

Imaging

bull Commonly fill 3rd

ventriclebull Diffusely enhance with

contrastbull Appear to infiltrate the

thalamusbull Large tumors may be

difficult to differentiatefrom suprasellar masses

10202016 46unusual giomas

10202016 47unusual giomas

bull Surgical management is limited by locationbull Access through the dialted ventricle and lamina

terminalis may be challangingbull Subtotal resection is thus commonbull Role of adjuvant radiotherapy is unknown

10202016 48unusual giomas

bull Acta Neurochir (Wien) 2008 Nov150(11)1183-91 doi 101007s00701-008-0014-6 Epub

2008 Oct 21

bull Chordoid glioma of the third ventricle

bull Vanhauwaert DJ1 Clement F Van Dorpe J Deruytter MJKGROUND

bull Chordoid glioma is a rare tumour (World Health Organisation grade II) originating from the third ventricle

with both glial and chordoid features It was first described by Brat in 1998 Since there is no detailed

information available on the outcome after surgery and adjuvant treatment we reviewed the literature

bull METHODS

bull A literature search through PUBMED revealed 50 cases of chordoid glioma Most reports

were found in pathology journals Information on the postoperative course was sometimes

very limited We reviewed the available literature and studied in detail the presenting

symptoms mortality and postoperative complications in relation to the extent of resective

surgery as well as the importance of adjuvant treatment

bull CONCLUSIONS

bull Mortality in the immediate postoperative period is 32 and is higher after gross total

resection as compared to subtotal resection Non-fatal postoperative complications are

hypothalamic disorders and mental alterations Gross total resection is the treatment of

choice since no recurrence has been reported after macroscopically complete resection

but this is often difficult because of the location and adherence to the hypothalamus The

role of postoperative radiotherapy is uncertain There is some indication that radiosurgery

with or without conventional irradiation is superior to conventional radiation alone Planned

subtotal resection followed by stereotactic radiosurgery can be a safe and effective

alternative in a patient in whom gross total resection is considered to be too risky

There is no report on the use of chemotherapy in the treatment of chordoid gliomas More

information about the optimal treatment strategy is needed and more reports are also needed10202016 49unusual giomas

bull Neurologist 2011 Jan17(1)52bull Chordoid glioma a case report and literature reviewbull Liu WP1 Cheng JX Yi XC Zhen HN Fei Z Li Q Zhang Xbull chordoid glioma is a rare tumor (World Health Organization grade II) with both

glial and chordoid features often located in the suprasellar region and anterior third ventricle It was first described by Brat in 1998 Because there is no detailed information available from the clinical perspective we reviewed the literature

bull METHODS

bull a literature search through PUBMED and CNKI revealed 64 cases of chordoid glioma Information on the clinical course was very limited We reviewed the literature and studied the pathologic and imaging features postoperative mortality and morbidity in relation to surgical extension and approaches and the importance of adjuvant treatment

bull CONCLUSIONS bull mortality in the immediate postoperative period is 28 and postoperative

morbidity is 60 which are statistically higher after gross total resection as compared with subtotal resection Translamina terminalis approach is considered to be the best approach The current study cannot document that patients have longer survival and higher quality of life after gross total resection than subtotal resection The role of postoperative radiotherapy is uncertain and there is no report on the use of chemotherapy More information about the optimal treatment strategy is needed

10202016 50unusual giomas

Conclusion

bull They are identified by their unique morphologicalfeatures

bull Many of these have indolent course and identified byepilepsy

bull Potentail diagnosis should be considered in youngpatients with seizures and cortically based lesions

bull Correct diagnosis requires experiencedneuropathologisrt

bull As more experienced is gained more knowledgetowards better understanding shall provide betteroutcomes in these patients

10202016 unusual giomas 51

10202016 52unusual giomas

Content supportndash youmann neurosurgery textbook

Subependymal Giant Cell Astrocytomabull WHO grade 1bull Generally occur in

patients with tuberoussclerosis

bull Typical location isintraventricular

bull Present with features ofraised ICP andobstructive HCP

bull TUBEROUS Sclerosis isan autosomal dominantdisease

bull Mental retardationseizures adenomassebaceum

bull Altered skinpigmentations retinaltumors subungualfibromas tumors ofpancreas and spleen

10202016 3unusual giomas

Pathologybull Rarely undiagnosedbull Found typically in

intraventricular locationsbull Typical appearance of giant

cells mixed with cells ofastrocyte lineage

bull Well demarcated tumorswith modest infilteartioninto surrounding whitematter

bull Giant cells with abundanteosinophilic cytoplasm andspindle shaped nuclei

bull Mitoses nuclear atypianecrosis endothelialproliferation withaggressive behaviour

bull Anaplastic transformationis rare

bull IHC marker show positivityfor GFAP AND S-100

10202016 4unusual giomas

Imaging

bull CT demonstratescalcification as well ascontrast enhancement

10202016 5unusual giomas

MRI

bull Isointense tohypointense on T1 massin the periventricularlocation

bull Hyperintense signal onT2 WI

bull Strongly enhancedsignals on post contrast

10202016 6unusual giomas

Management

bull Do not require interventionunless bolck the foramen ofmonroe

bull Primary treatment issurgical total resection

bull Subtotal resection may beadequate in case totalresection is hazardous

bull Tumor rarely becomesaggressive

bull Tarnscortical transcallosalapproach

bull If surgery not possible ordenied by pt thenmanagement of HCP byshunt

bull Role of chemotherapy hasshown promises

10202016 7unusual giomas

Everolimus for Subependymal Giant-Cell Astrocytomas in Tuberous SclerosisDarcy A Krueger MD PhD Marguerite M Care MD Katherine Holland MD PhD KarenAgricola FNP Cynthia Tudor PNP Prajakta Mangeshkar MS Kimberly A Wilson MSAnna Byars PhD Tarek Sahmoud MD PhD and David Neal Franz MDN Engl J Med 2010 3631801-181

bull Backgroundbull Neurosurgical resection is the standard treatment for subependymal giant-cell astrocytomas in patients with the tuberous

sclerosis complex An alternative may be the use of everolimus which inhibits the mammalian target of rapamycin a protein regulated by gene products involved in the tuberous sclerosis complex

bull Methodsbull Patients 3 years of age or older with serial growth of subependymal giant-cell astrocytomas were eligible for this open-label

study The primary efficacy end point was the change in volume of subependymal giant-cell astrocytomas between baseline and 6 months We gave everolimus orally at a dose of 30 mg per square meter of body-surface area to achieve a trough concentration of 5 to 15 ng per milliliter

bull Resultsbull We enrolled 28 patients Everolimus therapy was associated with a clinically meaningful reduction in volume of the

primary subependymal giant-cell astrocytoma as assessed on independent central review (Plt0001 for baseline vs 6 months) with a reduction of at least 30 in 21 patients (75) and at least 50 in 9 patients (32) Marked reductions were seen within 3 months and were sustained There were no new lesions worsening hydrocephalus evidence of increased intracranial pressure or necessity for surgical resection or other therapy for subependymal giant-cell astrocytoma Of the 16 patients for whom 24-hour video electroencephalography data were available seizure frequency for the 6-month study period (vs the previous 6-month period) decreased in 9 did not change in 6 and increased in 1 (median change minus1 seizure P=002) The mean (plusmnSD) score on the validated Quality-of-Life in Childhood Epilepsy questionnaire (on which scores can range from 0 to 100 with higher scores indicating a better quality of life) was improved at 3 months (634plusmn124) and 6 months (621plusmn142) over the baseline score (578plusmn140) Single cases of grade 3 treatment-related sinusitis pneumonia viral bronchitis tooth infection stomatitis and leukopenia were reported

bull Conclusionsbull Everolimus therapy was associated with marked reduction in the volume of subependymal giant-cell

astrocytomas and seizure frequency and may be a potential alternative to neurosurgical resection in some cases though long-term studies are needed

10202016 8unusual giomas

Angiocentric glioma

bull Described by WHO in2005

bull Initially included withastroblastoma andchoroid glioma

bull Mean age at diagnosis is17 years

bull Pts present with longhistory of seizures

bull Microscopically show themonomorphous bipolarcells associated withnormal vessels of thecortex and white matter

bull Tumor cells are spindleshaped

bull Mitoses are rarebull Positive for S-100 GFAP

vimentin

10202016 9unusual giomas

Imaging

bull CT images show it as ahypodense lesion at thecortex

bull MR shows a mass withstalk towards theventricular surfacemdashpathognomic

bull Hypointense to iso onT1WI

bull Hyperintense on T2WIbull Post contrast

enhancement is sparse

10202016 10unusual giomas

10202016 11unusual giomas

Management and outcome

bull Follow an indolent coursebull Rare progressionbull Surgical cure obtained by total resectionbull Aggressive forms are exceedingly rare

10202016 12unusual giomas

bull Perm J 2013 Winter17(1)e100-2 doi 107812TPP12-060

bull The treatment of angiocentric glioma case report and literature review

bull Alexandru D1 Haghighi B Muhonen MG

bull Abstract

bull Angiocentric glioma is a recently described tumor recognized since 2007

by the World Health Organization Classification of Tumours of the Central

Nervous System We present the only case of angiocentric glioma at our

institution in the last 15 years and review the literature in an attempt to

establish prognostic parameters Our search revealed only 27 cases of

angiocentric glioma in the literature The most common presenting

symptom of angiocentric glioma was seizures Gross total resection of

the lesion was curative without need for radiation or chemotherapy

10202016 13unusual giomas

Astroblastoma

bull WHO grade 1bull Initially described by Bailey

and Bucy in 1930bull Has been a matter of debate

for similar features asanaplastic astrocytomaglioblastoma andgemistocytic astrocytomas

bull Commonly found in 1st

three decades of lifebull Female

preponderance(111)

bull Affects the hemispheresinvolving cortex subcortical andperiventricular regions

bull No infratentorail tumorhave been reported

bull Pts present with features ofraised ICP cortical deficitsseizures personalitychanges

10202016 14unusual giomas

bull Greatly variable naturalhistory

bull Have a slow andindolent course

bull Rarely show anaggressive outcome

bull Almost always definedby their grossappearance

bull Cut surface revealhomogenous soft pinkgrey substances

bull Cystic areas arefrequently encountered

bull The clusters of cellsform pseudorossette

10202016 15unusual giomas

bull Richly supplied byblood vessels

bull Nuclei of tumor cellsare away from bloodvessels and send fineprocesse to the vesselwall

bull High number of mitoticfigures can be found

bull Cellular atypia isfrequently noted

bull On IHC tumor cellsstain positive for GFAP

bull Positivity for vimentinNSE S-1OO EMA

bull These are positive foranti leu 7 antibodiesspecific for cells ofepithelial origin

bull Central necrosis can befound

10202016 16unusual giomas

Imaging

bull On CT the appearnacesvaries from poorlydefined hypodensetumor to welldemarcated and highlyenhancing masses

10202016 17unusual giomas

bull On MR image tumorsare hypointense towhite matter on T1

bull Hyperintense on T2bull Hyperintense on FLAIRbull Enhancement is seen

around the cystic centre

10202016 18unusual giomas

Management And Prognosis

bull Surgical resection is main treatmentbull Gross total resection can be easily achieved as

lesions are mostly in the cortical areasbull Therapeutic value is of radiation and

chemotherapy is uncertain as these are raretumors

bull Prediction of outcome is difficult as these remainindolent for a long time and may rapidlytransform to glioblastoma resulting in fataloutcomes

10202016 19unusual giomas

Pilomyxoid astrocytoma

bull Recently described tumorsbull Similarities with pilocytic

astrocytomasbull Until recently these were

grouped with PAbull These involve entire

neuraxisbull Mean age at preentation is

18 monthsbull Most are found in

hypothalamic ndash chiasmicregion

bull Pts present with features ofraised ICP

bull RAISED HEADCIRCUMFERENCE may beonly subtle sign in infants

bull Histologically consists ofmyxoid matrix with cells inloose fibrillary and myxoidbackground

bull Tumor cells formperivascular rosettes

10202016 20unusual giomas

bull Preferred sites includebull (1) optic nerve (lsquooptic

nerve gliomarsquo)bull (2) optic

chiasmhypothalamusbull (3) thalamus and basal

gangliabull (4) cerebral hemispherebull (5) cerebellum (lsquocerebellar

astrocytomarsquo) andbull (6) brainstem (dorsal

exophytic brainstemglioma)

bull Genetically chracterized bythe presence of NF1mutations BRAFduplications and theabsence of IDH1 mutation

10202016 21unusual giomas

Imaging

bull The huge chiasmatic-hypothalamic tumor revealslow signal intensity on aT1-weighted image (A)

bull Bright signal intensity on aT2-weighted image (B)

bull Gadolinium-enhancedbrain MR images showheterogeneously strongenhancement (C and D)

bull Leptomeningealdisseminations at thecervical spine levels

10202016 22unusual giomas

bull NO standard careprotocols have beendefined

bull As these are newlydefined and raretumors

bull Surgical resection isfavoured if locationfavours

bull Mean progression freesurvival is 25 months

bull Mean overall survival is60 months

bull Reduced survival isbecause of inability toachieve gross resection

10202016 23unusual giomas

bull The usual indications foradjuvant treatment(chemotherapy orradiation therapy)include tumor recurrenceafter initial completeresection or symptomatictumors

bull In addition adjuvanttreatment is generallyindicated for tumors withgrowth on follow-upimaging even in theabsence of symptoms

bull The role of chemotherapyis in evolution

bull Chemotherapy may beimplemented for the treatment ofinoperable or partially resectedgliomas

bull The decision to use postoperativeadjuvant therapy also depends ona lesions perceived malignanttendencies as the potentialbenefits of radiation therapy andchemotherapy must be weighedagainst their morbidity

bull The decision to use postoperativeadjuvant therapy also depends ona lesions perceived malignanttendencies as the potentialbenefits of radiation therapy andchemotherapy must be weighedagainst their morbidity

10202016 24unusual giomas

Pleomorphic xanthoastrocytoma

bull First described by Kepsand colleuges in 1979

bull Represents lt1 of allastrocytomas

bull May be found alongentire neuraxis mostlysupratentorial( temporallobe)

bull Cerebellum and soinalcord are the other sites

bull Pts present in 2nd and 3rd

decadesbull Median age at 14 yearsbull Both sexes are affected

equallybull 70-80 pts present with

seizures and headachefocal neuro deficits

bull Rarely tumor mayhemmorrhage

10202016 25unusual giomas

bull Supratentorail are mostoften cortical and meningesbased

bull Chronic seizuresbull Grossely these are firm

tumors variable in colorrelatively avascular

bull Typically invade the piaarachnoid space

bull 13 pts have involvementof all the three meninges

bull Cyst fluid is xanthochromic

bull Very specific microscpoiccriteria for diagnosis are

1 Pleomorphic andxanthomatous astrocytes

2 Perivascular lymphocytes3 Non infilterating histology4 Reticulin stainingbull Pleomorphism can be as

vast as glioblastoma orsarcomatous tumors

10202016 26unusual giomas

bull IHC Staining revealsspindle cells and roundpleomorphic cellspositive for S-100 andGFAP

bull Staining forsynaptophysin and NSEmay be also present

bull Some workers havereported them to be asubtype of ganglioglioma

bull Association between theNeurofibromatosis Sturge Weber Syndrome

bull Loss of chr 9 and gain ofchromosome 7 have beenfound

10202016 27unusual giomas

Imaging

bull CTbull PXAs are typically hypo

or isodense and may bewell or poorlydemarcated usually withno or little surroundingoedema

bull Calcification is rarebull Due to its superficial

location it may causescalloping of theoverlying bone

10202016 28unusual giomas

bull MRIbull T1

ndash solid component iso tohypointense cf grey matter

ndash cystic component low signalndash leptomeningeal involvement

seen in over 70 of casesbull T1 C+ (Gd)

ndash solid component usuallyenhances vividly

bull T2ndash solid component iso to

hyperintense cf grey matterndash cystic component high signalndash on T2 FLAIR sequence cystic

areas show hyperintensityrelative to CSF due to higherprotein contents

ndash little surrounding vasogenicoedema

10202016 29unusual giomas

Management amp Outcome

bull Quite rare tumorsbull Surgical excision appears to

be best favoured modalitybull Actuarial survival trates are

91 at 5 years and 82 at10 years

bull Median survival 18 years

bull Total resection should beattempted in every case asfar as possible

bull Resection of cyst lining isnot indicated

bull Data regarding the use ofadjuvant therapy at thistime are limited to few casereports only

bull Pts who had incompleteresection high mitoticindex necrosis should bereserved for strict vigilanceand CEMRI on follow ups

10202016 30unusual giomas

bull At progression repeatresection be attempted

bull Proression should betaken as aggressivetumor type

bull Malignanttransformation maynecessitate adjuvanttherapy

10202016 31unusual giomas

bull Br J Neurosurg 2013 Dec27(6)759-64 doi 103109026886972013776666 Epub 2013 Mar 20

bull Pleomorphic xanthoastrocytoma long-term results of surgical treatment and analysis of prognosticfactors

bull Gallo P1 Cecchi PC Locatelli F Rizzo P Ghimenton C Gerosa M Pinna G

bull BACKGROUND

bull Pleomorphic Xanthoastrocytoma (PXA) is a rare brain tumour most commonly affecting children andyoung adults To date only few data regarding the long-term follow-up of these patients after surgery are

available The aim of this study is to describe our single-institution experience in the surgicalmanagement of this particular glioma over a period of over 18 years

bull METHODS

bull We performed a retrospective review of all cases of PXA (40 patients) operated upon at theDepartment of Neurosurgery of Verona Italy between 1990 and 2008 The impact of clinical radiologicalsurgical and histological factors on overall survival (OS) and progression-free survival (PFS) was analysed bymeans of univariate and multivariate models

bull FINDINGS

bull We achieved a gross total resection (GTR) in 65 of patients Histological diagnosis was of grade II in 80anaplastic features were present in the remaining 20 Adjuvant treatment radiotherapy or chemo-

radiotherapy was administered in 40 of the cases Median follow-up was 74 months OS at 5- and10 years was 7632 and 6824 respectively PFS at 5- and 10 years was 71 and 58respectively In the multivariate model histological grade extent of resection and age at diagnosis (le 30years vs gt 30 years) were the only independent prognostic factors for both OS and PFS

bull CONCLUSIONS

bull Our retrospective long-term study confirms the relatively favourable prognosis associatedwith PXA Young patients with a low-grade tumour (WHO grade II) who underwent GTR carrythe longest OS and PFS

10202016 32unusual giomas

bull Brain Tumor Pathol 2006 Apr23(1)55-63

bull A case of anaplastic pleomorphic xanthoastrocytoma presenting with tumor bleeding and cerebrospinal fluid dissemination

bull Asano K1 Miyamoto S Kubo O Kikkukawa T Yagihashi A Ohkuma H

bull Pleomorphic xanthoastrocytoma (PXA) has been considered an astrocytic tumorwith a relatively favorable prognosis However PXA cases having several recurrent patterns with poor prognosis have been reported in recent years and a new concept of anaplastic PXA has been proposed The present case was a 59-year-old woman who presented with tumor bleeding onset and cerebrospinal fluid dissemination The patient had sudden-onset right hemiparesis aphasia and consciousness disturbance and was admitted to a local area hospital After emergency surgery had removed the hematoma postoperative contrast-enhanced CT scan revealed a left temporal tumor A second surgery was therefore performed for initial tumor removal 2 months later Histopathological findings showed that the tumor was typical PXA with strong pleomorphism and xanthomatous changes and contained an ependymoma-like component in the center area However endothelial proliferation and mitosis were more remarkable compared to ordinary PXA The MIB-1 labeling index was 98 high From these findings the histopathological diagnosis was anaplastic PXA The patient underwent surgery to remove recurrent tumors 5 and 16 months later The patient died 36 months after the first onset and CT revealed glioblastoma-like

findings and cerebrospinal fluid dissemination This case report is the first case in which PXA presented with tumor bleeding onset Histopathological findings suggested anaplastic PXA from the first surgical specimens and PXA recurred many times We thus believe that the patient displayed primary anaplastic PXA rather than secondary anaplastic PXA that results in malignant transformation10202016 33unusual giomas

Ganglioglioma

bull WHO grade 1bull Initially described by

Perkins in 1926bull Benign well

differentiatedneuroepithelial tumors

bull Most commonly found intemporal lobe though canoccur anywhere in CNS

bull Commonly found inyoung patients withepilepsy

bull Most commonly notedtumor in temporal lobeepilepsy patients

bull Have been described inbrain steem spinal cordand cerebellum

bull Account for 1 ofprimary brain tumors

10202016 34unusual giomas

bull Male dominencebull Most common features

are seizures headachedizziness ataxiaprogressive weakness

bull Malignanttransformation is rare

bull Histology showsimmature mixture ofabnormal neural andglial elements

bull Macroscopically appearsolid or cystic

bull Cystic are welldelineatedfromsurrounding brain

bull Calcification is common

10202016 35unusual giomas

bull IHC staing shows reactivity for GFAP S-100 andvimentin

bull Neurons show synaptophysin class III betatubulin NFP chromogranin reactivity

10202016 36unusual giomas

Imaging

bull Highly variable imagingcharacters

bull No special typicalimaging criteria

bull Enhancement patternsare highly variable

bull Iso to hypointense onT1WI

bull Heterogenous andhyperintense on T2WI

bull Can contain solid cyticcalcified components

10202016 37unusual giomas

bull FDG PET scan showshypometabolism oreumetabolism in thesepatients

10202016 38unusual giomas

Management And Outcome

bull Gold standard is grosstotal resection

bull Seizure control aftersurgery is as high as80

bull Survival rates are highbull Event free survival of

low grade and highgrade gangiogioma are57 vs 15

bull The optimal surgicalresection depends uponthe eloquent areasinvolved in the vicinityof the tumor

bull The use ofchemotherapy is notwell established

bull Typically they shouldbe allowed for chemoand radiotherapy

10202016 39unusual giomas

bull The use of adjuvant therapy is warranted in thosecases which harbour residual lesions or features ofanaplasia

bull Potential harm to nearby brain areas preclude thejudicious use of radiation

10202016 40unusual giomas

bull Brain Tumor Res Treat 2014 Oct 2(2) 49ndash55

bull Treatment and Outcomes for Gangliogliomas A Single-Center Review of 16 Patients

bull Jye Young Song Jeong Hoon Kim Young Hyun Cho Chang Jin Kim and Eun Jung Lee

bull Ganglioglioma is a rare and slowly growing benign tumor We investigated the outcomes of patients who underwent different combination treatments

bull Methods

bull Between 1998 and 2012 16 patients including 11 men and 5 women with a median age of 125 years (range 25-65 years) were treated for intracranial gangliogliomas at our institution The median follow-up period was 57 years (range 48 days-156 years) Fifteen cases were included in the outcome assessment because one patient was lost to follow-up Complete resection was achieved in 8 (53) patients Six (40) patients underwent incomplete resection with or without adjuvant radiotherapy and one patient with a brainstem tumor underwent only stereotactic biopsy

bull Results

bull Gangliogliomas predominantly affected young (875) male patients and most frequently presented with seizures (64) Of eight patients who underwent complete resection seven did not show recurrence whereas only three of six with incomplete resection showed no recurrence Four patients with recurrence received salvage treatments (two repeat surgeries and two radiosurgeries) A tumor control rate of 93 (1415) was achieved at the last follow-up No recurrence or malignant changes were observed after a median follow-up of 12 and 45 years in four patients who received gamma knife (GK) radiosurgery as adjuvant and salvage treatment

bull Conclusion

bull Complete resection produced the best outcomes and incomplete resection followed by adjuvant or salvage treatments showed favorable outcomes In patients who are not eligible for complete resection because of tumor location or potential neurologic deficits following surgery GK radiosurgery should be considered for the treatment of residual or recurrent tumors

10202016 41unusual giomas

Papillary tumours of pineal

bull WHO grade II AND IIIbull Relatively new edition to WHObull They are equal in both sexesbull Reach medical attention due to headache mental

status changes and altered visionbull Contain eosinophilic cells with distinctive

borders with large pleomorphic nucleibull Positive for cytokeratin synaptophysin stain

variably for GFAP vimentin S-100

10202016 unusual giomas 42

Imaging

bull Generally found to be diffusely enhancinglesion of pineal region

bull Post 3rd ventricle with HCPbull Appear well demarcated from thalamus and

cerebellum

10202016 unusual giomas 43

Mangement

bull Standard treatment is excisionbull Grow slowly and donot transform to

malignant tumorsbull Local recurrence is however possiblebull Radiotherapy appears to provide good control

in local recurrence

10202016 unusual giomas 44

Choroid Glioma Of Third Ventricle

bull WHO Grade IIbull Ist recognised by WHO in

1998bull Arise in the anterior third

ventriclebull Commonly come to

attention after HCPbull Appera to contain in 3rd

ventricle and thought toarise from ventricularsurface

bull Composed of GFAP andvimentin positive cells inmucinous matrix

bull Nuclei are uniform insize and shape

bull Indolent growthbull May show EGF receptor

positivitybull Some suggets these tumor

arise from the lminaterminalis andventricular tanycytes

10202016 45unusual giomas

Imaging

bull Commonly fill 3rd

ventriclebull Diffusely enhance with

contrastbull Appear to infiltrate the

thalamusbull Large tumors may be

difficult to differentiatefrom suprasellar masses

10202016 46unusual giomas

10202016 47unusual giomas

bull Surgical management is limited by locationbull Access through the dialted ventricle and lamina

terminalis may be challangingbull Subtotal resection is thus commonbull Role of adjuvant radiotherapy is unknown

10202016 48unusual giomas

bull Acta Neurochir (Wien) 2008 Nov150(11)1183-91 doi 101007s00701-008-0014-6 Epub

2008 Oct 21

bull Chordoid glioma of the third ventricle

bull Vanhauwaert DJ1 Clement F Van Dorpe J Deruytter MJKGROUND

bull Chordoid glioma is a rare tumour (World Health Organisation grade II) originating from the third ventricle

with both glial and chordoid features It was first described by Brat in 1998 Since there is no detailed

information available on the outcome after surgery and adjuvant treatment we reviewed the literature

bull METHODS

bull A literature search through PUBMED revealed 50 cases of chordoid glioma Most reports

were found in pathology journals Information on the postoperative course was sometimes

very limited We reviewed the available literature and studied in detail the presenting

symptoms mortality and postoperative complications in relation to the extent of resective

surgery as well as the importance of adjuvant treatment

bull CONCLUSIONS

bull Mortality in the immediate postoperative period is 32 and is higher after gross total

resection as compared to subtotal resection Non-fatal postoperative complications are

hypothalamic disorders and mental alterations Gross total resection is the treatment of

choice since no recurrence has been reported after macroscopically complete resection

but this is often difficult because of the location and adherence to the hypothalamus The

role of postoperative radiotherapy is uncertain There is some indication that radiosurgery

with or without conventional irradiation is superior to conventional radiation alone Planned

subtotal resection followed by stereotactic radiosurgery can be a safe and effective

alternative in a patient in whom gross total resection is considered to be too risky

There is no report on the use of chemotherapy in the treatment of chordoid gliomas More

information about the optimal treatment strategy is needed and more reports are also needed10202016 49unusual giomas

bull Neurologist 2011 Jan17(1)52bull Chordoid glioma a case report and literature reviewbull Liu WP1 Cheng JX Yi XC Zhen HN Fei Z Li Q Zhang Xbull chordoid glioma is a rare tumor (World Health Organization grade II) with both

glial and chordoid features often located in the suprasellar region and anterior third ventricle It was first described by Brat in 1998 Because there is no detailed information available from the clinical perspective we reviewed the literature

bull METHODS

bull a literature search through PUBMED and CNKI revealed 64 cases of chordoid glioma Information on the clinical course was very limited We reviewed the literature and studied the pathologic and imaging features postoperative mortality and morbidity in relation to surgical extension and approaches and the importance of adjuvant treatment

bull CONCLUSIONS bull mortality in the immediate postoperative period is 28 and postoperative

morbidity is 60 which are statistically higher after gross total resection as compared with subtotal resection Translamina terminalis approach is considered to be the best approach The current study cannot document that patients have longer survival and higher quality of life after gross total resection than subtotal resection The role of postoperative radiotherapy is uncertain and there is no report on the use of chemotherapy More information about the optimal treatment strategy is needed

10202016 50unusual giomas

Conclusion

bull They are identified by their unique morphologicalfeatures

bull Many of these have indolent course and identified byepilepsy

bull Potentail diagnosis should be considered in youngpatients with seizures and cortically based lesions

bull Correct diagnosis requires experiencedneuropathologisrt

bull As more experienced is gained more knowledgetowards better understanding shall provide betteroutcomes in these patients

10202016 unusual giomas 51

10202016 52unusual giomas

Content supportndash youmann neurosurgery textbook

Pathologybull Rarely undiagnosedbull Found typically in

intraventricular locationsbull Typical appearance of giant

cells mixed with cells ofastrocyte lineage

bull Well demarcated tumorswith modest infilteartioninto surrounding whitematter

bull Giant cells with abundanteosinophilic cytoplasm andspindle shaped nuclei

bull Mitoses nuclear atypianecrosis endothelialproliferation withaggressive behaviour

bull Anaplastic transformationis rare

bull IHC marker show positivityfor GFAP AND S-100

10202016 4unusual giomas

Imaging

bull CT demonstratescalcification as well ascontrast enhancement

10202016 5unusual giomas

MRI

bull Isointense tohypointense on T1 massin the periventricularlocation

bull Hyperintense signal onT2 WI

bull Strongly enhancedsignals on post contrast

10202016 6unusual giomas

Management

bull Do not require interventionunless bolck the foramen ofmonroe

bull Primary treatment issurgical total resection

bull Subtotal resection may beadequate in case totalresection is hazardous

bull Tumor rarely becomesaggressive

bull Tarnscortical transcallosalapproach

bull If surgery not possible ordenied by pt thenmanagement of HCP byshunt

bull Role of chemotherapy hasshown promises

10202016 7unusual giomas

Everolimus for Subependymal Giant-Cell Astrocytomas in Tuberous SclerosisDarcy A Krueger MD PhD Marguerite M Care MD Katherine Holland MD PhD KarenAgricola FNP Cynthia Tudor PNP Prajakta Mangeshkar MS Kimberly A Wilson MSAnna Byars PhD Tarek Sahmoud MD PhD and David Neal Franz MDN Engl J Med 2010 3631801-181

bull Backgroundbull Neurosurgical resection is the standard treatment for subependymal giant-cell astrocytomas in patients with the tuberous

sclerosis complex An alternative may be the use of everolimus which inhibits the mammalian target of rapamycin a protein regulated by gene products involved in the tuberous sclerosis complex

bull Methodsbull Patients 3 years of age or older with serial growth of subependymal giant-cell astrocytomas were eligible for this open-label

study The primary efficacy end point was the change in volume of subependymal giant-cell astrocytomas between baseline and 6 months We gave everolimus orally at a dose of 30 mg per square meter of body-surface area to achieve a trough concentration of 5 to 15 ng per milliliter

bull Resultsbull We enrolled 28 patients Everolimus therapy was associated with a clinically meaningful reduction in volume of the

primary subependymal giant-cell astrocytoma as assessed on independent central review (Plt0001 for baseline vs 6 months) with a reduction of at least 30 in 21 patients (75) and at least 50 in 9 patients (32) Marked reductions were seen within 3 months and were sustained There were no new lesions worsening hydrocephalus evidence of increased intracranial pressure or necessity for surgical resection or other therapy for subependymal giant-cell astrocytoma Of the 16 patients for whom 24-hour video electroencephalography data were available seizure frequency for the 6-month study period (vs the previous 6-month period) decreased in 9 did not change in 6 and increased in 1 (median change minus1 seizure P=002) The mean (plusmnSD) score on the validated Quality-of-Life in Childhood Epilepsy questionnaire (on which scores can range from 0 to 100 with higher scores indicating a better quality of life) was improved at 3 months (634plusmn124) and 6 months (621plusmn142) over the baseline score (578plusmn140) Single cases of grade 3 treatment-related sinusitis pneumonia viral bronchitis tooth infection stomatitis and leukopenia were reported

bull Conclusionsbull Everolimus therapy was associated with marked reduction in the volume of subependymal giant-cell

astrocytomas and seizure frequency and may be a potential alternative to neurosurgical resection in some cases though long-term studies are needed

10202016 8unusual giomas

Angiocentric glioma

bull Described by WHO in2005

bull Initially included withastroblastoma andchoroid glioma

bull Mean age at diagnosis is17 years

bull Pts present with longhistory of seizures

bull Microscopically show themonomorphous bipolarcells associated withnormal vessels of thecortex and white matter

bull Tumor cells are spindleshaped

bull Mitoses are rarebull Positive for S-100 GFAP

vimentin

10202016 9unusual giomas

Imaging

bull CT images show it as ahypodense lesion at thecortex

bull MR shows a mass withstalk towards theventricular surfacemdashpathognomic

bull Hypointense to iso onT1WI

bull Hyperintense on T2WIbull Post contrast

enhancement is sparse

10202016 10unusual giomas

10202016 11unusual giomas

Management and outcome

bull Follow an indolent coursebull Rare progressionbull Surgical cure obtained by total resectionbull Aggressive forms are exceedingly rare

10202016 12unusual giomas

bull Perm J 2013 Winter17(1)e100-2 doi 107812TPP12-060

bull The treatment of angiocentric glioma case report and literature review

bull Alexandru D1 Haghighi B Muhonen MG

bull Abstract

bull Angiocentric glioma is a recently described tumor recognized since 2007

by the World Health Organization Classification of Tumours of the Central

Nervous System We present the only case of angiocentric glioma at our

institution in the last 15 years and review the literature in an attempt to

establish prognostic parameters Our search revealed only 27 cases of

angiocentric glioma in the literature The most common presenting

symptom of angiocentric glioma was seizures Gross total resection of

the lesion was curative without need for radiation or chemotherapy

10202016 13unusual giomas

Astroblastoma

bull WHO grade 1bull Initially described by Bailey

and Bucy in 1930bull Has been a matter of debate

for similar features asanaplastic astrocytomaglioblastoma andgemistocytic astrocytomas

bull Commonly found in 1st

three decades of lifebull Female

preponderance(111)

bull Affects the hemispheresinvolving cortex subcortical andperiventricular regions

bull No infratentorail tumorhave been reported

bull Pts present with features ofraised ICP cortical deficitsseizures personalitychanges

10202016 14unusual giomas

bull Greatly variable naturalhistory

bull Have a slow andindolent course

bull Rarely show anaggressive outcome

bull Almost always definedby their grossappearance

bull Cut surface revealhomogenous soft pinkgrey substances

bull Cystic areas arefrequently encountered

bull The clusters of cellsform pseudorossette

10202016 15unusual giomas

bull Richly supplied byblood vessels

bull Nuclei of tumor cellsare away from bloodvessels and send fineprocesse to the vesselwall

bull High number of mitoticfigures can be found

bull Cellular atypia isfrequently noted

bull On IHC tumor cellsstain positive for GFAP

bull Positivity for vimentinNSE S-1OO EMA

bull These are positive foranti leu 7 antibodiesspecific for cells ofepithelial origin

bull Central necrosis can befound

10202016 16unusual giomas

Imaging

bull On CT the appearnacesvaries from poorlydefined hypodensetumor to welldemarcated and highlyenhancing masses

10202016 17unusual giomas

bull On MR image tumorsare hypointense towhite matter on T1

bull Hyperintense on T2bull Hyperintense on FLAIRbull Enhancement is seen

around the cystic centre

10202016 18unusual giomas

Management And Prognosis

bull Surgical resection is main treatmentbull Gross total resection can be easily achieved as

lesions are mostly in the cortical areasbull Therapeutic value is of radiation and

chemotherapy is uncertain as these are raretumors

bull Prediction of outcome is difficult as these remainindolent for a long time and may rapidlytransform to glioblastoma resulting in fataloutcomes

10202016 19unusual giomas

Pilomyxoid astrocytoma

bull Recently described tumorsbull Similarities with pilocytic

astrocytomasbull Until recently these were

grouped with PAbull These involve entire

neuraxisbull Mean age at preentation is

18 monthsbull Most are found in

hypothalamic ndash chiasmicregion

bull Pts present with features ofraised ICP

bull RAISED HEADCIRCUMFERENCE may beonly subtle sign in infants

bull Histologically consists ofmyxoid matrix with cells inloose fibrillary and myxoidbackground

bull Tumor cells formperivascular rosettes

10202016 20unusual giomas

bull Preferred sites includebull (1) optic nerve (lsquooptic

nerve gliomarsquo)bull (2) optic

chiasmhypothalamusbull (3) thalamus and basal

gangliabull (4) cerebral hemispherebull (5) cerebellum (lsquocerebellar

astrocytomarsquo) andbull (6) brainstem (dorsal

exophytic brainstemglioma)

bull Genetically chracterized bythe presence of NF1mutations BRAFduplications and theabsence of IDH1 mutation

10202016 21unusual giomas

Imaging

bull The huge chiasmatic-hypothalamic tumor revealslow signal intensity on aT1-weighted image (A)

bull Bright signal intensity on aT2-weighted image (B)

bull Gadolinium-enhancedbrain MR images showheterogeneously strongenhancement (C and D)

bull Leptomeningealdisseminations at thecervical spine levels

10202016 22unusual giomas

bull NO standard careprotocols have beendefined

bull As these are newlydefined and raretumors

bull Surgical resection isfavoured if locationfavours

bull Mean progression freesurvival is 25 months

bull Mean overall survival is60 months

bull Reduced survival isbecause of inability toachieve gross resection

10202016 23unusual giomas

bull The usual indications foradjuvant treatment(chemotherapy orradiation therapy)include tumor recurrenceafter initial completeresection or symptomatictumors

bull In addition adjuvanttreatment is generallyindicated for tumors withgrowth on follow-upimaging even in theabsence of symptoms

bull The role of chemotherapyis in evolution

bull Chemotherapy may beimplemented for the treatment ofinoperable or partially resectedgliomas

bull The decision to use postoperativeadjuvant therapy also depends ona lesions perceived malignanttendencies as the potentialbenefits of radiation therapy andchemotherapy must be weighedagainst their morbidity

bull The decision to use postoperativeadjuvant therapy also depends ona lesions perceived malignanttendencies as the potentialbenefits of radiation therapy andchemotherapy must be weighedagainst their morbidity

10202016 24unusual giomas

Pleomorphic xanthoastrocytoma

bull First described by Kepsand colleuges in 1979

bull Represents lt1 of allastrocytomas

bull May be found alongentire neuraxis mostlysupratentorial( temporallobe)

bull Cerebellum and soinalcord are the other sites

bull Pts present in 2nd and 3rd

decadesbull Median age at 14 yearsbull Both sexes are affected

equallybull 70-80 pts present with

seizures and headachefocal neuro deficits

bull Rarely tumor mayhemmorrhage

10202016 25unusual giomas

bull Supratentorail are mostoften cortical and meningesbased

bull Chronic seizuresbull Grossely these are firm

tumors variable in colorrelatively avascular

bull Typically invade the piaarachnoid space

bull 13 pts have involvementof all the three meninges

bull Cyst fluid is xanthochromic

bull Very specific microscpoiccriteria for diagnosis are

1 Pleomorphic andxanthomatous astrocytes

2 Perivascular lymphocytes3 Non infilterating histology4 Reticulin stainingbull Pleomorphism can be as

vast as glioblastoma orsarcomatous tumors

10202016 26unusual giomas

bull IHC Staining revealsspindle cells and roundpleomorphic cellspositive for S-100 andGFAP

bull Staining forsynaptophysin and NSEmay be also present

bull Some workers havereported them to be asubtype of ganglioglioma

bull Association between theNeurofibromatosis Sturge Weber Syndrome

bull Loss of chr 9 and gain ofchromosome 7 have beenfound

10202016 27unusual giomas

Imaging

bull CTbull PXAs are typically hypo

or isodense and may bewell or poorlydemarcated usually withno or little surroundingoedema

bull Calcification is rarebull Due to its superficial

location it may causescalloping of theoverlying bone

10202016 28unusual giomas

bull MRIbull T1

ndash solid component iso tohypointense cf grey matter

ndash cystic component low signalndash leptomeningeal involvement

seen in over 70 of casesbull T1 C+ (Gd)

ndash solid component usuallyenhances vividly

bull T2ndash solid component iso to

hyperintense cf grey matterndash cystic component high signalndash on T2 FLAIR sequence cystic

areas show hyperintensityrelative to CSF due to higherprotein contents

ndash little surrounding vasogenicoedema

10202016 29unusual giomas

Management amp Outcome

bull Quite rare tumorsbull Surgical excision appears to

be best favoured modalitybull Actuarial survival trates are

91 at 5 years and 82 at10 years

bull Median survival 18 years

bull Total resection should beattempted in every case asfar as possible

bull Resection of cyst lining isnot indicated

bull Data regarding the use ofadjuvant therapy at thistime are limited to few casereports only

bull Pts who had incompleteresection high mitoticindex necrosis should bereserved for strict vigilanceand CEMRI on follow ups

10202016 30unusual giomas

bull At progression repeatresection be attempted

bull Proression should betaken as aggressivetumor type

bull Malignanttransformation maynecessitate adjuvanttherapy

10202016 31unusual giomas

bull Br J Neurosurg 2013 Dec27(6)759-64 doi 103109026886972013776666 Epub 2013 Mar 20

bull Pleomorphic xanthoastrocytoma long-term results of surgical treatment and analysis of prognosticfactors

bull Gallo P1 Cecchi PC Locatelli F Rizzo P Ghimenton C Gerosa M Pinna G

bull BACKGROUND

bull Pleomorphic Xanthoastrocytoma (PXA) is a rare brain tumour most commonly affecting children andyoung adults To date only few data regarding the long-term follow-up of these patients after surgery are

available The aim of this study is to describe our single-institution experience in the surgicalmanagement of this particular glioma over a period of over 18 years

bull METHODS

bull We performed a retrospective review of all cases of PXA (40 patients) operated upon at theDepartment of Neurosurgery of Verona Italy between 1990 and 2008 The impact of clinical radiologicalsurgical and histological factors on overall survival (OS) and progression-free survival (PFS) was analysed bymeans of univariate and multivariate models

bull FINDINGS

bull We achieved a gross total resection (GTR) in 65 of patients Histological diagnosis was of grade II in 80anaplastic features were present in the remaining 20 Adjuvant treatment radiotherapy or chemo-

radiotherapy was administered in 40 of the cases Median follow-up was 74 months OS at 5- and10 years was 7632 and 6824 respectively PFS at 5- and 10 years was 71 and 58respectively In the multivariate model histological grade extent of resection and age at diagnosis (le 30years vs gt 30 years) were the only independent prognostic factors for both OS and PFS

bull CONCLUSIONS

bull Our retrospective long-term study confirms the relatively favourable prognosis associatedwith PXA Young patients with a low-grade tumour (WHO grade II) who underwent GTR carrythe longest OS and PFS

10202016 32unusual giomas

bull Brain Tumor Pathol 2006 Apr23(1)55-63

bull A case of anaplastic pleomorphic xanthoastrocytoma presenting with tumor bleeding and cerebrospinal fluid dissemination

bull Asano K1 Miyamoto S Kubo O Kikkukawa T Yagihashi A Ohkuma H

bull Pleomorphic xanthoastrocytoma (PXA) has been considered an astrocytic tumorwith a relatively favorable prognosis However PXA cases having several recurrent patterns with poor prognosis have been reported in recent years and a new concept of anaplastic PXA has been proposed The present case was a 59-year-old woman who presented with tumor bleeding onset and cerebrospinal fluid dissemination The patient had sudden-onset right hemiparesis aphasia and consciousness disturbance and was admitted to a local area hospital After emergency surgery had removed the hematoma postoperative contrast-enhanced CT scan revealed a left temporal tumor A second surgery was therefore performed for initial tumor removal 2 months later Histopathological findings showed that the tumor was typical PXA with strong pleomorphism and xanthomatous changes and contained an ependymoma-like component in the center area However endothelial proliferation and mitosis were more remarkable compared to ordinary PXA The MIB-1 labeling index was 98 high From these findings the histopathological diagnosis was anaplastic PXA The patient underwent surgery to remove recurrent tumors 5 and 16 months later The patient died 36 months after the first onset and CT revealed glioblastoma-like

findings and cerebrospinal fluid dissemination This case report is the first case in which PXA presented with tumor bleeding onset Histopathological findings suggested anaplastic PXA from the first surgical specimens and PXA recurred many times We thus believe that the patient displayed primary anaplastic PXA rather than secondary anaplastic PXA that results in malignant transformation10202016 33unusual giomas

Ganglioglioma

bull WHO grade 1bull Initially described by

Perkins in 1926bull Benign well

differentiatedneuroepithelial tumors

bull Most commonly found intemporal lobe though canoccur anywhere in CNS

bull Commonly found inyoung patients withepilepsy

bull Most commonly notedtumor in temporal lobeepilepsy patients

bull Have been described inbrain steem spinal cordand cerebellum

bull Account for 1 ofprimary brain tumors

10202016 34unusual giomas

bull Male dominencebull Most common features

are seizures headachedizziness ataxiaprogressive weakness

bull Malignanttransformation is rare

bull Histology showsimmature mixture ofabnormal neural andglial elements

bull Macroscopically appearsolid or cystic

bull Cystic are welldelineatedfromsurrounding brain

bull Calcification is common

10202016 35unusual giomas

bull IHC staing shows reactivity for GFAP S-100 andvimentin

bull Neurons show synaptophysin class III betatubulin NFP chromogranin reactivity

10202016 36unusual giomas

Imaging

bull Highly variable imagingcharacters

bull No special typicalimaging criteria

bull Enhancement patternsare highly variable

bull Iso to hypointense onT1WI

bull Heterogenous andhyperintense on T2WI

bull Can contain solid cyticcalcified components

10202016 37unusual giomas

bull FDG PET scan showshypometabolism oreumetabolism in thesepatients

10202016 38unusual giomas

Management And Outcome

bull Gold standard is grosstotal resection

bull Seizure control aftersurgery is as high as80

bull Survival rates are highbull Event free survival of

low grade and highgrade gangiogioma are57 vs 15

bull The optimal surgicalresection depends uponthe eloquent areasinvolved in the vicinityof the tumor

bull The use ofchemotherapy is notwell established

bull Typically they shouldbe allowed for chemoand radiotherapy

10202016 39unusual giomas

bull The use of adjuvant therapy is warranted in thosecases which harbour residual lesions or features ofanaplasia

bull Potential harm to nearby brain areas preclude thejudicious use of radiation

10202016 40unusual giomas

bull Brain Tumor Res Treat 2014 Oct 2(2) 49ndash55

bull Treatment and Outcomes for Gangliogliomas A Single-Center Review of 16 Patients

bull Jye Young Song Jeong Hoon Kim Young Hyun Cho Chang Jin Kim and Eun Jung Lee

bull Ganglioglioma is a rare and slowly growing benign tumor We investigated the outcomes of patients who underwent different combination treatments

bull Methods

bull Between 1998 and 2012 16 patients including 11 men and 5 women with a median age of 125 years (range 25-65 years) were treated for intracranial gangliogliomas at our institution The median follow-up period was 57 years (range 48 days-156 years) Fifteen cases were included in the outcome assessment because one patient was lost to follow-up Complete resection was achieved in 8 (53) patients Six (40) patients underwent incomplete resection with or without adjuvant radiotherapy and one patient with a brainstem tumor underwent only stereotactic biopsy

bull Results

bull Gangliogliomas predominantly affected young (875) male patients and most frequently presented with seizures (64) Of eight patients who underwent complete resection seven did not show recurrence whereas only three of six with incomplete resection showed no recurrence Four patients with recurrence received salvage treatments (two repeat surgeries and two radiosurgeries) A tumor control rate of 93 (1415) was achieved at the last follow-up No recurrence or malignant changes were observed after a median follow-up of 12 and 45 years in four patients who received gamma knife (GK) radiosurgery as adjuvant and salvage treatment

bull Conclusion

bull Complete resection produced the best outcomes and incomplete resection followed by adjuvant or salvage treatments showed favorable outcomes In patients who are not eligible for complete resection because of tumor location or potential neurologic deficits following surgery GK radiosurgery should be considered for the treatment of residual or recurrent tumors

10202016 41unusual giomas

Papillary tumours of pineal

bull WHO grade II AND IIIbull Relatively new edition to WHObull They are equal in both sexesbull Reach medical attention due to headache mental

status changes and altered visionbull Contain eosinophilic cells with distinctive

borders with large pleomorphic nucleibull Positive for cytokeratin synaptophysin stain

variably for GFAP vimentin S-100

10202016 unusual giomas 42

Imaging

bull Generally found to be diffusely enhancinglesion of pineal region

bull Post 3rd ventricle with HCPbull Appear well demarcated from thalamus and

cerebellum

10202016 unusual giomas 43

Mangement

bull Standard treatment is excisionbull Grow slowly and donot transform to

malignant tumorsbull Local recurrence is however possiblebull Radiotherapy appears to provide good control

in local recurrence

10202016 unusual giomas 44

Choroid Glioma Of Third Ventricle

bull WHO Grade IIbull Ist recognised by WHO in

1998bull Arise in the anterior third

ventriclebull Commonly come to

attention after HCPbull Appera to contain in 3rd

ventricle and thought toarise from ventricularsurface

bull Composed of GFAP andvimentin positive cells inmucinous matrix

bull Nuclei are uniform insize and shape

bull Indolent growthbull May show EGF receptor

positivitybull Some suggets these tumor

arise from the lminaterminalis andventricular tanycytes

10202016 45unusual giomas

Imaging

bull Commonly fill 3rd

ventriclebull Diffusely enhance with

contrastbull Appear to infiltrate the

thalamusbull Large tumors may be

difficult to differentiatefrom suprasellar masses

10202016 46unusual giomas

10202016 47unusual giomas

bull Surgical management is limited by locationbull Access through the dialted ventricle and lamina

terminalis may be challangingbull Subtotal resection is thus commonbull Role of adjuvant radiotherapy is unknown

10202016 48unusual giomas

bull Acta Neurochir (Wien) 2008 Nov150(11)1183-91 doi 101007s00701-008-0014-6 Epub

2008 Oct 21

bull Chordoid glioma of the third ventricle

bull Vanhauwaert DJ1 Clement F Van Dorpe J Deruytter MJKGROUND

bull Chordoid glioma is a rare tumour (World Health Organisation grade II) originating from the third ventricle

with both glial and chordoid features It was first described by Brat in 1998 Since there is no detailed

information available on the outcome after surgery and adjuvant treatment we reviewed the literature

bull METHODS

bull A literature search through PUBMED revealed 50 cases of chordoid glioma Most reports

were found in pathology journals Information on the postoperative course was sometimes

very limited We reviewed the available literature and studied in detail the presenting

symptoms mortality and postoperative complications in relation to the extent of resective

surgery as well as the importance of adjuvant treatment

bull CONCLUSIONS

bull Mortality in the immediate postoperative period is 32 and is higher after gross total

resection as compared to subtotal resection Non-fatal postoperative complications are

hypothalamic disorders and mental alterations Gross total resection is the treatment of

choice since no recurrence has been reported after macroscopically complete resection

but this is often difficult because of the location and adherence to the hypothalamus The

role of postoperative radiotherapy is uncertain There is some indication that radiosurgery

with or without conventional irradiation is superior to conventional radiation alone Planned

subtotal resection followed by stereotactic radiosurgery can be a safe and effective

alternative in a patient in whom gross total resection is considered to be too risky

There is no report on the use of chemotherapy in the treatment of chordoid gliomas More

information about the optimal treatment strategy is needed and more reports are also needed10202016 49unusual giomas

bull Neurologist 2011 Jan17(1)52bull Chordoid glioma a case report and literature reviewbull Liu WP1 Cheng JX Yi XC Zhen HN Fei Z Li Q Zhang Xbull chordoid glioma is a rare tumor (World Health Organization grade II) with both

glial and chordoid features often located in the suprasellar region and anterior third ventricle It was first described by Brat in 1998 Because there is no detailed information available from the clinical perspective we reviewed the literature

bull METHODS

bull a literature search through PUBMED and CNKI revealed 64 cases of chordoid glioma Information on the clinical course was very limited We reviewed the literature and studied the pathologic and imaging features postoperative mortality and morbidity in relation to surgical extension and approaches and the importance of adjuvant treatment

bull CONCLUSIONS bull mortality in the immediate postoperative period is 28 and postoperative

morbidity is 60 which are statistically higher after gross total resection as compared with subtotal resection Translamina terminalis approach is considered to be the best approach The current study cannot document that patients have longer survival and higher quality of life after gross total resection than subtotal resection The role of postoperative radiotherapy is uncertain and there is no report on the use of chemotherapy More information about the optimal treatment strategy is needed

10202016 50unusual giomas

Conclusion

bull They are identified by their unique morphologicalfeatures

bull Many of these have indolent course and identified byepilepsy

bull Potentail diagnosis should be considered in youngpatients with seizures and cortically based lesions

bull Correct diagnosis requires experiencedneuropathologisrt

bull As more experienced is gained more knowledgetowards better understanding shall provide betteroutcomes in these patients

10202016 unusual giomas 51

10202016 52unusual giomas

Content supportndash youmann neurosurgery textbook

Imaging

bull CT demonstratescalcification as well ascontrast enhancement

10202016 5unusual giomas

MRI

bull Isointense tohypointense on T1 massin the periventricularlocation

bull Hyperintense signal onT2 WI

bull Strongly enhancedsignals on post contrast

10202016 6unusual giomas

Management

bull Do not require interventionunless bolck the foramen ofmonroe

bull Primary treatment issurgical total resection

bull Subtotal resection may beadequate in case totalresection is hazardous

bull Tumor rarely becomesaggressive

bull Tarnscortical transcallosalapproach

bull If surgery not possible ordenied by pt thenmanagement of HCP byshunt

bull Role of chemotherapy hasshown promises

10202016 7unusual giomas

Everolimus for Subependymal Giant-Cell Astrocytomas in Tuberous SclerosisDarcy A Krueger MD PhD Marguerite M Care MD Katherine Holland MD PhD KarenAgricola FNP Cynthia Tudor PNP Prajakta Mangeshkar MS Kimberly A Wilson MSAnna Byars PhD Tarek Sahmoud MD PhD and David Neal Franz MDN Engl J Med 2010 3631801-181

bull Backgroundbull Neurosurgical resection is the standard treatment for subependymal giant-cell astrocytomas in patients with the tuberous

sclerosis complex An alternative may be the use of everolimus which inhibits the mammalian target of rapamycin a protein regulated by gene products involved in the tuberous sclerosis complex

bull Methodsbull Patients 3 years of age or older with serial growth of subependymal giant-cell astrocytomas were eligible for this open-label

study The primary efficacy end point was the change in volume of subependymal giant-cell astrocytomas between baseline and 6 months We gave everolimus orally at a dose of 30 mg per square meter of body-surface area to achieve a trough concentration of 5 to 15 ng per milliliter

bull Resultsbull We enrolled 28 patients Everolimus therapy was associated with a clinically meaningful reduction in volume of the

primary subependymal giant-cell astrocytoma as assessed on independent central review (Plt0001 for baseline vs 6 months) with a reduction of at least 30 in 21 patients (75) and at least 50 in 9 patients (32) Marked reductions were seen within 3 months and were sustained There were no new lesions worsening hydrocephalus evidence of increased intracranial pressure or necessity for surgical resection or other therapy for subependymal giant-cell astrocytoma Of the 16 patients for whom 24-hour video electroencephalography data were available seizure frequency for the 6-month study period (vs the previous 6-month period) decreased in 9 did not change in 6 and increased in 1 (median change minus1 seizure P=002) The mean (plusmnSD) score on the validated Quality-of-Life in Childhood Epilepsy questionnaire (on which scores can range from 0 to 100 with higher scores indicating a better quality of life) was improved at 3 months (634plusmn124) and 6 months (621plusmn142) over the baseline score (578plusmn140) Single cases of grade 3 treatment-related sinusitis pneumonia viral bronchitis tooth infection stomatitis and leukopenia were reported

bull Conclusionsbull Everolimus therapy was associated with marked reduction in the volume of subependymal giant-cell

astrocytomas and seizure frequency and may be a potential alternative to neurosurgical resection in some cases though long-term studies are needed

10202016 8unusual giomas

Angiocentric glioma

bull Described by WHO in2005

bull Initially included withastroblastoma andchoroid glioma

bull Mean age at diagnosis is17 years

bull Pts present with longhistory of seizures

bull Microscopically show themonomorphous bipolarcells associated withnormal vessels of thecortex and white matter

bull Tumor cells are spindleshaped

bull Mitoses are rarebull Positive for S-100 GFAP

vimentin

10202016 9unusual giomas

Imaging

bull CT images show it as ahypodense lesion at thecortex

bull MR shows a mass withstalk towards theventricular surfacemdashpathognomic

bull Hypointense to iso onT1WI

bull Hyperintense on T2WIbull Post contrast

enhancement is sparse

10202016 10unusual giomas

10202016 11unusual giomas

Management and outcome

bull Follow an indolent coursebull Rare progressionbull Surgical cure obtained by total resectionbull Aggressive forms are exceedingly rare

10202016 12unusual giomas

bull Perm J 2013 Winter17(1)e100-2 doi 107812TPP12-060

bull The treatment of angiocentric glioma case report and literature review

bull Alexandru D1 Haghighi B Muhonen MG

bull Abstract

bull Angiocentric glioma is a recently described tumor recognized since 2007

by the World Health Organization Classification of Tumours of the Central

Nervous System We present the only case of angiocentric glioma at our

institution in the last 15 years and review the literature in an attempt to

establish prognostic parameters Our search revealed only 27 cases of

angiocentric glioma in the literature The most common presenting

symptom of angiocentric glioma was seizures Gross total resection of

the lesion was curative without need for radiation or chemotherapy

10202016 13unusual giomas

Astroblastoma

bull WHO grade 1bull Initially described by Bailey

and Bucy in 1930bull Has been a matter of debate

for similar features asanaplastic astrocytomaglioblastoma andgemistocytic astrocytomas

bull Commonly found in 1st

three decades of lifebull Female

preponderance(111)

bull Affects the hemispheresinvolving cortex subcortical andperiventricular regions

bull No infratentorail tumorhave been reported

bull Pts present with features ofraised ICP cortical deficitsseizures personalitychanges

10202016 14unusual giomas

bull Greatly variable naturalhistory

bull Have a slow andindolent course

bull Rarely show anaggressive outcome

bull Almost always definedby their grossappearance

bull Cut surface revealhomogenous soft pinkgrey substances

bull Cystic areas arefrequently encountered

bull The clusters of cellsform pseudorossette

10202016 15unusual giomas

bull Richly supplied byblood vessels

bull Nuclei of tumor cellsare away from bloodvessels and send fineprocesse to the vesselwall

bull High number of mitoticfigures can be found

bull Cellular atypia isfrequently noted

bull On IHC tumor cellsstain positive for GFAP

bull Positivity for vimentinNSE S-1OO EMA

bull These are positive foranti leu 7 antibodiesspecific for cells ofepithelial origin

bull Central necrosis can befound

10202016 16unusual giomas

Imaging

bull On CT the appearnacesvaries from poorlydefined hypodensetumor to welldemarcated and highlyenhancing masses

10202016 17unusual giomas

bull On MR image tumorsare hypointense towhite matter on T1

bull Hyperintense on T2bull Hyperintense on FLAIRbull Enhancement is seen

around the cystic centre

10202016 18unusual giomas

Management And Prognosis

bull Surgical resection is main treatmentbull Gross total resection can be easily achieved as

lesions are mostly in the cortical areasbull Therapeutic value is of radiation and

chemotherapy is uncertain as these are raretumors

bull Prediction of outcome is difficult as these remainindolent for a long time and may rapidlytransform to glioblastoma resulting in fataloutcomes

10202016 19unusual giomas

Pilomyxoid astrocytoma

bull Recently described tumorsbull Similarities with pilocytic

astrocytomasbull Until recently these were

grouped with PAbull These involve entire

neuraxisbull Mean age at preentation is

18 monthsbull Most are found in

hypothalamic ndash chiasmicregion

bull Pts present with features ofraised ICP

bull RAISED HEADCIRCUMFERENCE may beonly subtle sign in infants

bull Histologically consists ofmyxoid matrix with cells inloose fibrillary and myxoidbackground

bull Tumor cells formperivascular rosettes

10202016 20unusual giomas

bull Preferred sites includebull (1) optic nerve (lsquooptic

nerve gliomarsquo)bull (2) optic

chiasmhypothalamusbull (3) thalamus and basal

gangliabull (4) cerebral hemispherebull (5) cerebellum (lsquocerebellar

astrocytomarsquo) andbull (6) brainstem (dorsal

exophytic brainstemglioma)

bull Genetically chracterized bythe presence of NF1mutations BRAFduplications and theabsence of IDH1 mutation

10202016 21unusual giomas

Imaging

bull The huge chiasmatic-hypothalamic tumor revealslow signal intensity on aT1-weighted image (A)

bull Bright signal intensity on aT2-weighted image (B)

bull Gadolinium-enhancedbrain MR images showheterogeneously strongenhancement (C and D)

bull Leptomeningealdisseminations at thecervical spine levels

10202016 22unusual giomas

bull NO standard careprotocols have beendefined

bull As these are newlydefined and raretumors

bull Surgical resection isfavoured if locationfavours

bull Mean progression freesurvival is 25 months

bull Mean overall survival is60 months

bull Reduced survival isbecause of inability toachieve gross resection

10202016 23unusual giomas

bull The usual indications foradjuvant treatment(chemotherapy orradiation therapy)include tumor recurrenceafter initial completeresection or symptomatictumors

bull In addition adjuvanttreatment is generallyindicated for tumors withgrowth on follow-upimaging even in theabsence of symptoms

bull The role of chemotherapyis in evolution

bull Chemotherapy may beimplemented for the treatment ofinoperable or partially resectedgliomas

bull The decision to use postoperativeadjuvant therapy also depends ona lesions perceived malignanttendencies as the potentialbenefits of radiation therapy andchemotherapy must be weighedagainst their morbidity

bull The decision to use postoperativeadjuvant therapy also depends ona lesions perceived malignanttendencies as the potentialbenefits of radiation therapy andchemotherapy must be weighedagainst their morbidity

10202016 24unusual giomas

Pleomorphic xanthoastrocytoma

bull First described by Kepsand colleuges in 1979

bull Represents lt1 of allastrocytomas

bull May be found alongentire neuraxis mostlysupratentorial( temporallobe)

bull Cerebellum and soinalcord are the other sites

bull Pts present in 2nd and 3rd

decadesbull Median age at 14 yearsbull Both sexes are affected

equallybull 70-80 pts present with

seizures and headachefocal neuro deficits

bull Rarely tumor mayhemmorrhage

10202016 25unusual giomas

bull Supratentorail are mostoften cortical and meningesbased

bull Chronic seizuresbull Grossely these are firm

tumors variable in colorrelatively avascular

bull Typically invade the piaarachnoid space

bull 13 pts have involvementof all the three meninges

bull Cyst fluid is xanthochromic

bull Very specific microscpoiccriteria for diagnosis are

1 Pleomorphic andxanthomatous astrocytes

2 Perivascular lymphocytes3 Non infilterating histology4 Reticulin stainingbull Pleomorphism can be as

vast as glioblastoma orsarcomatous tumors

10202016 26unusual giomas

bull IHC Staining revealsspindle cells and roundpleomorphic cellspositive for S-100 andGFAP

bull Staining forsynaptophysin and NSEmay be also present

bull Some workers havereported them to be asubtype of ganglioglioma

bull Association between theNeurofibromatosis Sturge Weber Syndrome

bull Loss of chr 9 and gain ofchromosome 7 have beenfound

10202016 27unusual giomas

Imaging

bull CTbull PXAs are typically hypo

or isodense and may bewell or poorlydemarcated usually withno or little surroundingoedema

bull Calcification is rarebull Due to its superficial

location it may causescalloping of theoverlying bone

10202016 28unusual giomas

bull MRIbull T1

ndash solid component iso tohypointense cf grey matter

ndash cystic component low signalndash leptomeningeal involvement

seen in over 70 of casesbull T1 C+ (Gd)

ndash solid component usuallyenhances vividly

bull T2ndash solid component iso to

hyperintense cf grey matterndash cystic component high signalndash on T2 FLAIR sequence cystic

areas show hyperintensityrelative to CSF due to higherprotein contents

ndash little surrounding vasogenicoedema

10202016 29unusual giomas

Management amp Outcome

bull Quite rare tumorsbull Surgical excision appears to

be best favoured modalitybull Actuarial survival trates are

91 at 5 years and 82 at10 years

bull Median survival 18 years

bull Total resection should beattempted in every case asfar as possible

bull Resection of cyst lining isnot indicated

bull Data regarding the use ofadjuvant therapy at thistime are limited to few casereports only

bull Pts who had incompleteresection high mitoticindex necrosis should bereserved for strict vigilanceand CEMRI on follow ups

10202016 30unusual giomas

bull At progression repeatresection be attempted

bull Proression should betaken as aggressivetumor type

bull Malignanttransformation maynecessitate adjuvanttherapy

10202016 31unusual giomas

bull Br J Neurosurg 2013 Dec27(6)759-64 doi 103109026886972013776666 Epub 2013 Mar 20

bull Pleomorphic xanthoastrocytoma long-term results of surgical treatment and analysis of prognosticfactors

bull Gallo P1 Cecchi PC Locatelli F Rizzo P Ghimenton C Gerosa M Pinna G

bull BACKGROUND

bull Pleomorphic Xanthoastrocytoma (PXA) is a rare brain tumour most commonly affecting children andyoung adults To date only few data regarding the long-term follow-up of these patients after surgery are

available The aim of this study is to describe our single-institution experience in the surgicalmanagement of this particular glioma over a period of over 18 years

bull METHODS

bull We performed a retrospective review of all cases of PXA (40 patients) operated upon at theDepartment of Neurosurgery of Verona Italy between 1990 and 2008 The impact of clinical radiologicalsurgical and histological factors on overall survival (OS) and progression-free survival (PFS) was analysed bymeans of univariate and multivariate models

bull FINDINGS

bull We achieved a gross total resection (GTR) in 65 of patients Histological diagnosis was of grade II in 80anaplastic features were present in the remaining 20 Adjuvant treatment radiotherapy or chemo-

radiotherapy was administered in 40 of the cases Median follow-up was 74 months OS at 5- and10 years was 7632 and 6824 respectively PFS at 5- and 10 years was 71 and 58respectively In the multivariate model histological grade extent of resection and age at diagnosis (le 30years vs gt 30 years) were the only independent prognostic factors for both OS and PFS

bull CONCLUSIONS

bull Our retrospective long-term study confirms the relatively favourable prognosis associatedwith PXA Young patients with a low-grade tumour (WHO grade II) who underwent GTR carrythe longest OS and PFS

10202016 32unusual giomas

bull Brain Tumor Pathol 2006 Apr23(1)55-63

bull A case of anaplastic pleomorphic xanthoastrocytoma presenting with tumor bleeding and cerebrospinal fluid dissemination

bull Asano K1 Miyamoto S Kubo O Kikkukawa T Yagihashi A Ohkuma H

bull Pleomorphic xanthoastrocytoma (PXA) has been considered an astrocytic tumorwith a relatively favorable prognosis However PXA cases having several recurrent patterns with poor prognosis have been reported in recent years and a new concept of anaplastic PXA has been proposed The present case was a 59-year-old woman who presented with tumor bleeding onset and cerebrospinal fluid dissemination The patient had sudden-onset right hemiparesis aphasia and consciousness disturbance and was admitted to a local area hospital After emergency surgery had removed the hematoma postoperative contrast-enhanced CT scan revealed a left temporal tumor A second surgery was therefore performed for initial tumor removal 2 months later Histopathological findings showed that the tumor was typical PXA with strong pleomorphism and xanthomatous changes and contained an ependymoma-like component in the center area However endothelial proliferation and mitosis were more remarkable compared to ordinary PXA The MIB-1 labeling index was 98 high From these findings the histopathological diagnosis was anaplastic PXA The patient underwent surgery to remove recurrent tumors 5 and 16 months later The patient died 36 months after the first onset and CT revealed glioblastoma-like

findings and cerebrospinal fluid dissemination This case report is the first case in which PXA presented with tumor bleeding onset Histopathological findings suggested anaplastic PXA from the first surgical specimens and PXA recurred many times We thus believe that the patient displayed primary anaplastic PXA rather than secondary anaplastic PXA that results in malignant transformation10202016 33unusual giomas

Ganglioglioma

bull WHO grade 1bull Initially described by

Perkins in 1926bull Benign well

differentiatedneuroepithelial tumors

bull Most commonly found intemporal lobe though canoccur anywhere in CNS

bull Commonly found inyoung patients withepilepsy

bull Most commonly notedtumor in temporal lobeepilepsy patients

bull Have been described inbrain steem spinal cordand cerebellum

bull Account for 1 ofprimary brain tumors

10202016 34unusual giomas

bull Male dominencebull Most common features

are seizures headachedizziness ataxiaprogressive weakness

bull Malignanttransformation is rare

bull Histology showsimmature mixture ofabnormal neural andglial elements

bull Macroscopically appearsolid or cystic

bull Cystic are welldelineatedfromsurrounding brain

bull Calcification is common

10202016 35unusual giomas

bull IHC staing shows reactivity for GFAP S-100 andvimentin

bull Neurons show synaptophysin class III betatubulin NFP chromogranin reactivity

10202016 36unusual giomas

Imaging

bull Highly variable imagingcharacters

bull No special typicalimaging criteria

bull Enhancement patternsare highly variable

bull Iso to hypointense onT1WI

bull Heterogenous andhyperintense on T2WI

bull Can contain solid cyticcalcified components

10202016 37unusual giomas

bull FDG PET scan showshypometabolism oreumetabolism in thesepatients

10202016 38unusual giomas

Management And Outcome

bull Gold standard is grosstotal resection

bull Seizure control aftersurgery is as high as80

bull Survival rates are highbull Event free survival of

low grade and highgrade gangiogioma are57 vs 15

bull The optimal surgicalresection depends uponthe eloquent areasinvolved in the vicinityof the tumor

bull The use ofchemotherapy is notwell established

bull Typically they shouldbe allowed for chemoand radiotherapy

10202016 39unusual giomas

bull The use of adjuvant therapy is warranted in thosecases which harbour residual lesions or features ofanaplasia

bull Potential harm to nearby brain areas preclude thejudicious use of radiation

10202016 40unusual giomas

bull Brain Tumor Res Treat 2014 Oct 2(2) 49ndash55

bull Treatment and Outcomes for Gangliogliomas A Single-Center Review of 16 Patients

bull Jye Young Song Jeong Hoon Kim Young Hyun Cho Chang Jin Kim and Eun Jung Lee

bull Ganglioglioma is a rare and slowly growing benign tumor We investigated the outcomes of patients who underwent different combination treatments

bull Methods

bull Between 1998 and 2012 16 patients including 11 men and 5 women with a median age of 125 years (range 25-65 years) were treated for intracranial gangliogliomas at our institution The median follow-up period was 57 years (range 48 days-156 years) Fifteen cases were included in the outcome assessment because one patient was lost to follow-up Complete resection was achieved in 8 (53) patients Six (40) patients underwent incomplete resection with or without adjuvant radiotherapy and one patient with a brainstem tumor underwent only stereotactic biopsy

bull Results

bull Gangliogliomas predominantly affected young (875) male patients and most frequently presented with seizures (64) Of eight patients who underwent complete resection seven did not show recurrence whereas only three of six with incomplete resection showed no recurrence Four patients with recurrence received salvage treatments (two repeat surgeries and two radiosurgeries) A tumor control rate of 93 (1415) was achieved at the last follow-up No recurrence or malignant changes were observed after a median follow-up of 12 and 45 years in four patients who received gamma knife (GK) radiosurgery as adjuvant and salvage treatment

bull Conclusion

bull Complete resection produced the best outcomes and incomplete resection followed by adjuvant or salvage treatments showed favorable outcomes In patients who are not eligible for complete resection because of tumor location or potential neurologic deficits following surgery GK radiosurgery should be considered for the treatment of residual or recurrent tumors

10202016 41unusual giomas

Papillary tumours of pineal

bull WHO grade II AND IIIbull Relatively new edition to WHObull They are equal in both sexesbull Reach medical attention due to headache mental

status changes and altered visionbull Contain eosinophilic cells with distinctive

borders with large pleomorphic nucleibull Positive for cytokeratin synaptophysin stain

variably for GFAP vimentin S-100

10202016 unusual giomas 42

Imaging

bull Generally found to be diffusely enhancinglesion of pineal region

bull Post 3rd ventricle with HCPbull Appear well demarcated from thalamus and

cerebellum

10202016 unusual giomas 43

Mangement

bull Standard treatment is excisionbull Grow slowly and donot transform to

malignant tumorsbull Local recurrence is however possiblebull Radiotherapy appears to provide good control

in local recurrence

10202016 unusual giomas 44

Choroid Glioma Of Third Ventricle

bull WHO Grade IIbull Ist recognised by WHO in

1998bull Arise in the anterior third

ventriclebull Commonly come to

attention after HCPbull Appera to contain in 3rd

ventricle and thought toarise from ventricularsurface

bull Composed of GFAP andvimentin positive cells inmucinous matrix

bull Nuclei are uniform insize and shape

bull Indolent growthbull May show EGF receptor

positivitybull Some suggets these tumor

arise from the lminaterminalis andventricular tanycytes

10202016 45unusual giomas

Imaging

bull Commonly fill 3rd

ventriclebull Diffusely enhance with

contrastbull Appear to infiltrate the

thalamusbull Large tumors may be

difficult to differentiatefrom suprasellar masses

10202016 46unusual giomas

10202016 47unusual giomas

bull Surgical management is limited by locationbull Access through the dialted ventricle and lamina

terminalis may be challangingbull Subtotal resection is thus commonbull Role of adjuvant radiotherapy is unknown

10202016 48unusual giomas

bull Acta Neurochir (Wien) 2008 Nov150(11)1183-91 doi 101007s00701-008-0014-6 Epub

2008 Oct 21

bull Chordoid glioma of the third ventricle

bull Vanhauwaert DJ1 Clement F Van Dorpe J Deruytter MJKGROUND

bull Chordoid glioma is a rare tumour (World Health Organisation grade II) originating from the third ventricle

with both glial and chordoid features It was first described by Brat in 1998 Since there is no detailed

information available on the outcome after surgery and adjuvant treatment we reviewed the literature

bull METHODS

bull A literature search through PUBMED revealed 50 cases of chordoid glioma Most reports

were found in pathology journals Information on the postoperative course was sometimes

very limited We reviewed the available literature and studied in detail the presenting

symptoms mortality and postoperative complications in relation to the extent of resective

surgery as well as the importance of adjuvant treatment

bull CONCLUSIONS

bull Mortality in the immediate postoperative period is 32 and is higher after gross total

resection as compared to subtotal resection Non-fatal postoperative complications are

hypothalamic disorders and mental alterations Gross total resection is the treatment of

choice since no recurrence has been reported after macroscopically complete resection

but this is often difficult because of the location and adherence to the hypothalamus The

role of postoperative radiotherapy is uncertain There is some indication that radiosurgery

with or without conventional irradiation is superior to conventional radiation alone Planned

subtotal resection followed by stereotactic radiosurgery can be a safe and effective

alternative in a patient in whom gross total resection is considered to be too risky

There is no report on the use of chemotherapy in the treatment of chordoid gliomas More

information about the optimal treatment strategy is needed and more reports are also needed10202016 49unusual giomas

bull Neurologist 2011 Jan17(1)52bull Chordoid glioma a case report and literature reviewbull Liu WP1 Cheng JX Yi XC Zhen HN Fei Z Li Q Zhang Xbull chordoid glioma is a rare tumor (World Health Organization grade II) with both

glial and chordoid features often located in the suprasellar region and anterior third ventricle It was first described by Brat in 1998 Because there is no detailed information available from the clinical perspective we reviewed the literature

bull METHODS

bull a literature search through PUBMED and CNKI revealed 64 cases of chordoid glioma Information on the clinical course was very limited We reviewed the literature and studied the pathologic and imaging features postoperative mortality and morbidity in relation to surgical extension and approaches and the importance of adjuvant treatment

bull CONCLUSIONS bull mortality in the immediate postoperative period is 28 and postoperative

morbidity is 60 which are statistically higher after gross total resection as compared with subtotal resection Translamina terminalis approach is considered to be the best approach The current study cannot document that patients have longer survival and higher quality of life after gross total resection than subtotal resection The role of postoperative radiotherapy is uncertain and there is no report on the use of chemotherapy More information about the optimal treatment strategy is needed

10202016 50unusual giomas

Conclusion

bull They are identified by their unique morphologicalfeatures

bull Many of these have indolent course and identified byepilepsy

bull Potentail diagnosis should be considered in youngpatients with seizures and cortically based lesions

bull Correct diagnosis requires experiencedneuropathologisrt

bull As more experienced is gained more knowledgetowards better understanding shall provide betteroutcomes in these patients

10202016 unusual giomas 51

10202016 52unusual giomas

Content supportndash youmann neurosurgery textbook

MRI

bull Isointense tohypointense on T1 massin the periventricularlocation

bull Hyperintense signal onT2 WI

bull Strongly enhancedsignals on post contrast

10202016 6unusual giomas

Management

bull Do not require interventionunless bolck the foramen ofmonroe

bull Primary treatment issurgical total resection

bull Subtotal resection may beadequate in case totalresection is hazardous

bull Tumor rarely becomesaggressive

bull Tarnscortical transcallosalapproach

bull If surgery not possible ordenied by pt thenmanagement of HCP byshunt

bull Role of chemotherapy hasshown promises

10202016 7unusual giomas

Everolimus for Subependymal Giant-Cell Astrocytomas in Tuberous SclerosisDarcy A Krueger MD PhD Marguerite M Care MD Katherine Holland MD PhD KarenAgricola FNP Cynthia Tudor PNP Prajakta Mangeshkar MS Kimberly A Wilson MSAnna Byars PhD Tarek Sahmoud MD PhD and David Neal Franz MDN Engl J Med 2010 3631801-181

bull Backgroundbull Neurosurgical resection is the standard treatment for subependymal giant-cell astrocytomas in patients with the tuberous

sclerosis complex An alternative may be the use of everolimus which inhibits the mammalian target of rapamycin a protein regulated by gene products involved in the tuberous sclerosis complex

bull Methodsbull Patients 3 years of age or older with serial growth of subependymal giant-cell astrocytomas were eligible for this open-label

study The primary efficacy end point was the change in volume of subependymal giant-cell astrocytomas between baseline and 6 months We gave everolimus orally at a dose of 30 mg per square meter of body-surface area to achieve a trough concentration of 5 to 15 ng per milliliter

bull Resultsbull We enrolled 28 patients Everolimus therapy was associated with a clinically meaningful reduction in volume of the

primary subependymal giant-cell astrocytoma as assessed on independent central review (Plt0001 for baseline vs 6 months) with a reduction of at least 30 in 21 patients (75) and at least 50 in 9 patients (32) Marked reductions were seen within 3 months and were sustained There were no new lesions worsening hydrocephalus evidence of increased intracranial pressure or necessity for surgical resection or other therapy for subependymal giant-cell astrocytoma Of the 16 patients for whom 24-hour video electroencephalography data were available seizure frequency for the 6-month study period (vs the previous 6-month period) decreased in 9 did not change in 6 and increased in 1 (median change minus1 seizure P=002) The mean (plusmnSD) score on the validated Quality-of-Life in Childhood Epilepsy questionnaire (on which scores can range from 0 to 100 with higher scores indicating a better quality of life) was improved at 3 months (634plusmn124) and 6 months (621plusmn142) over the baseline score (578plusmn140) Single cases of grade 3 treatment-related sinusitis pneumonia viral bronchitis tooth infection stomatitis and leukopenia were reported

bull Conclusionsbull Everolimus therapy was associated with marked reduction in the volume of subependymal giant-cell

astrocytomas and seizure frequency and may be a potential alternative to neurosurgical resection in some cases though long-term studies are needed

10202016 8unusual giomas

Angiocentric glioma

bull Described by WHO in2005

bull Initially included withastroblastoma andchoroid glioma

bull Mean age at diagnosis is17 years

bull Pts present with longhistory of seizures

bull Microscopically show themonomorphous bipolarcells associated withnormal vessels of thecortex and white matter

bull Tumor cells are spindleshaped

bull Mitoses are rarebull Positive for S-100 GFAP

vimentin

10202016 9unusual giomas

Imaging

bull CT images show it as ahypodense lesion at thecortex

bull MR shows a mass withstalk towards theventricular surfacemdashpathognomic

bull Hypointense to iso onT1WI

bull Hyperintense on T2WIbull Post contrast

enhancement is sparse

10202016 10unusual giomas

10202016 11unusual giomas

Management and outcome

bull Follow an indolent coursebull Rare progressionbull Surgical cure obtained by total resectionbull Aggressive forms are exceedingly rare

10202016 12unusual giomas

bull Perm J 2013 Winter17(1)e100-2 doi 107812TPP12-060

bull The treatment of angiocentric glioma case report and literature review

bull Alexandru D1 Haghighi B Muhonen MG

bull Abstract

bull Angiocentric glioma is a recently described tumor recognized since 2007

by the World Health Organization Classification of Tumours of the Central

Nervous System We present the only case of angiocentric glioma at our

institution in the last 15 years and review the literature in an attempt to

establish prognostic parameters Our search revealed only 27 cases of

angiocentric glioma in the literature The most common presenting

symptom of angiocentric glioma was seizures Gross total resection of

the lesion was curative without need for radiation or chemotherapy

10202016 13unusual giomas

Astroblastoma

bull WHO grade 1bull Initially described by Bailey

and Bucy in 1930bull Has been a matter of debate

for similar features asanaplastic astrocytomaglioblastoma andgemistocytic astrocytomas

bull Commonly found in 1st

three decades of lifebull Female

preponderance(111)

bull Affects the hemispheresinvolving cortex subcortical andperiventricular regions

bull No infratentorail tumorhave been reported

bull Pts present with features ofraised ICP cortical deficitsseizures personalitychanges

10202016 14unusual giomas

bull Greatly variable naturalhistory

bull Have a slow andindolent course

bull Rarely show anaggressive outcome

bull Almost always definedby their grossappearance

bull Cut surface revealhomogenous soft pinkgrey substances

bull Cystic areas arefrequently encountered

bull The clusters of cellsform pseudorossette

10202016 15unusual giomas

bull Richly supplied byblood vessels

bull Nuclei of tumor cellsare away from bloodvessels and send fineprocesse to the vesselwall

bull High number of mitoticfigures can be found

bull Cellular atypia isfrequently noted

bull On IHC tumor cellsstain positive for GFAP

bull Positivity for vimentinNSE S-1OO EMA

bull These are positive foranti leu 7 antibodiesspecific for cells ofepithelial origin

bull Central necrosis can befound

10202016 16unusual giomas

Imaging

bull On CT the appearnacesvaries from poorlydefined hypodensetumor to welldemarcated and highlyenhancing masses

10202016 17unusual giomas

bull On MR image tumorsare hypointense towhite matter on T1

bull Hyperintense on T2bull Hyperintense on FLAIRbull Enhancement is seen

around the cystic centre

10202016 18unusual giomas

Management And Prognosis

bull Surgical resection is main treatmentbull Gross total resection can be easily achieved as

lesions are mostly in the cortical areasbull Therapeutic value is of radiation and

chemotherapy is uncertain as these are raretumors

bull Prediction of outcome is difficult as these remainindolent for a long time and may rapidlytransform to glioblastoma resulting in fataloutcomes

10202016 19unusual giomas

Pilomyxoid astrocytoma

bull Recently described tumorsbull Similarities with pilocytic

astrocytomasbull Until recently these were

grouped with PAbull These involve entire

neuraxisbull Mean age at preentation is

18 monthsbull Most are found in

hypothalamic ndash chiasmicregion

bull Pts present with features ofraised ICP

bull RAISED HEADCIRCUMFERENCE may beonly subtle sign in infants

bull Histologically consists ofmyxoid matrix with cells inloose fibrillary and myxoidbackground

bull Tumor cells formperivascular rosettes

10202016 20unusual giomas

bull Preferred sites includebull (1) optic nerve (lsquooptic

nerve gliomarsquo)bull (2) optic

chiasmhypothalamusbull (3) thalamus and basal

gangliabull (4) cerebral hemispherebull (5) cerebellum (lsquocerebellar

astrocytomarsquo) andbull (6) brainstem (dorsal

exophytic brainstemglioma)

bull Genetically chracterized bythe presence of NF1mutations BRAFduplications and theabsence of IDH1 mutation

10202016 21unusual giomas

Imaging

bull The huge chiasmatic-hypothalamic tumor revealslow signal intensity on aT1-weighted image (A)

bull Bright signal intensity on aT2-weighted image (B)

bull Gadolinium-enhancedbrain MR images showheterogeneously strongenhancement (C and D)

bull Leptomeningealdisseminations at thecervical spine levels

10202016 22unusual giomas

bull NO standard careprotocols have beendefined

bull As these are newlydefined and raretumors

bull Surgical resection isfavoured if locationfavours

bull Mean progression freesurvival is 25 months

bull Mean overall survival is60 months

bull Reduced survival isbecause of inability toachieve gross resection

10202016 23unusual giomas

bull The usual indications foradjuvant treatment(chemotherapy orradiation therapy)include tumor recurrenceafter initial completeresection or symptomatictumors

bull In addition adjuvanttreatment is generallyindicated for tumors withgrowth on follow-upimaging even in theabsence of symptoms

bull The role of chemotherapyis in evolution

bull Chemotherapy may beimplemented for the treatment ofinoperable or partially resectedgliomas

bull The decision to use postoperativeadjuvant therapy also depends ona lesions perceived malignanttendencies as the potentialbenefits of radiation therapy andchemotherapy must be weighedagainst their morbidity

bull The decision to use postoperativeadjuvant therapy also depends ona lesions perceived malignanttendencies as the potentialbenefits of radiation therapy andchemotherapy must be weighedagainst their morbidity

10202016 24unusual giomas

Pleomorphic xanthoastrocytoma

bull First described by Kepsand colleuges in 1979

bull Represents lt1 of allastrocytomas

bull May be found alongentire neuraxis mostlysupratentorial( temporallobe)

bull Cerebellum and soinalcord are the other sites

bull Pts present in 2nd and 3rd

decadesbull Median age at 14 yearsbull Both sexes are affected

equallybull 70-80 pts present with

seizures and headachefocal neuro deficits

bull Rarely tumor mayhemmorrhage

10202016 25unusual giomas

bull Supratentorail are mostoften cortical and meningesbased

bull Chronic seizuresbull Grossely these are firm

tumors variable in colorrelatively avascular

bull Typically invade the piaarachnoid space

bull 13 pts have involvementof all the three meninges

bull Cyst fluid is xanthochromic

bull Very specific microscpoiccriteria for diagnosis are

1 Pleomorphic andxanthomatous astrocytes

2 Perivascular lymphocytes3 Non infilterating histology4 Reticulin stainingbull Pleomorphism can be as

vast as glioblastoma orsarcomatous tumors

10202016 26unusual giomas

bull IHC Staining revealsspindle cells and roundpleomorphic cellspositive for S-100 andGFAP

bull Staining forsynaptophysin and NSEmay be also present

bull Some workers havereported them to be asubtype of ganglioglioma

bull Association between theNeurofibromatosis Sturge Weber Syndrome

bull Loss of chr 9 and gain ofchromosome 7 have beenfound

10202016 27unusual giomas

Imaging

bull CTbull PXAs are typically hypo

or isodense and may bewell or poorlydemarcated usually withno or little surroundingoedema

bull Calcification is rarebull Due to its superficial

location it may causescalloping of theoverlying bone

10202016 28unusual giomas

bull MRIbull T1

ndash solid component iso tohypointense cf grey matter

ndash cystic component low signalndash leptomeningeal involvement

seen in over 70 of casesbull T1 C+ (Gd)

ndash solid component usuallyenhances vividly

bull T2ndash solid component iso to

hyperintense cf grey matterndash cystic component high signalndash on T2 FLAIR sequence cystic

areas show hyperintensityrelative to CSF due to higherprotein contents

ndash little surrounding vasogenicoedema

10202016 29unusual giomas

Management amp Outcome

bull Quite rare tumorsbull Surgical excision appears to

be best favoured modalitybull Actuarial survival trates are

91 at 5 years and 82 at10 years

bull Median survival 18 years

bull Total resection should beattempted in every case asfar as possible

bull Resection of cyst lining isnot indicated

bull Data regarding the use ofadjuvant therapy at thistime are limited to few casereports only

bull Pts who had incompleteresection high mitoticindex necrosis should bereserved for strict vigilanceand CEMRI on follow ups

10202016 30unusual giomas

bull At progression repeatresection be attempted

bull Proression should betaken as aggressivetumor type

bull Malignanttransformation maynecessitate adjuvanttherapy

10202016 31unusual giomas

bull Br J Neurosurg 2013 Dec27(6)759-64 doi 103109026886972013776666 Epub 2013 Mar 20

bull Pleomorphic xanthoastrocytoma long-term results of surgical treatment and analysis of prognosticfactors

bull Gallo P1 Cecchi PC Locatelli F Rizzo P Ghimenton C Gerosa M Pinna G

bull BACKGROUND

bull Pleomorphic Xanthoastrocytoma (PXA) is a rare brain tumour most commonly affecting children andyoung adults To date only few data regarding the long-term follow-up of these patients after surgery are

available The aim of this study is to describe our single-institution experience in the surgicalmanagement of this particular glioma over a period of over 18 years

bull METHODS

bull We performed a retrospective review of all cases of PXA (40 patients) operated upon at theDepartment of Neurosurgery of Verona Italy between 1990 and 2008 The impact of clinical radiologicalsurgical and histological factors on overall survival (OS) and progression-free survival (PFS) was analysed bymeans of univariate and multivariate models

bull FINDINGS

bull We achieved a gross total resection (GTR) in 65 of patients Histological diagnosis was of grade II in 80anaplastic features were present in the remaining 20 Adjuvant treatment radiotherapy or chemo-

radiotherapy was administered in 40 of the cases Median follow-up was 74 months OS at 5- and10 years was 7632 and 6824 respectively PFS at 5- and 10 years was 71 and 58respectively In the multivariate model histological grade extent of resection and age at diagnosis (le 30years vs gt 30 years) were the only independent prognostic factors for both OS and PFS

bull CONCLUSIONS

bull Our retrospective long-term study confirms the relatively favourable prognosis associatedwith PXA Young patients with a low-grade tumour (WHO grade II) who underwent GTR carrythe longest OS and PFS

10202016 32unusual giomas

bull Brain Tumor Pathol 2006 Apr23(1)55-63

bull A case of anaplastic pleomorphic xanthoastrocytoma presenting with tumor bleeding and cerebrospinal fluid dissemination

bull Asano K1 Miyamoto S Kubo O Kikkukawa T Yagihashi A Ohkuma H

bull Pleomorphic xanthoastrocytoma (PXA) has been considered an astrocytic tumorwith a relatively favorable prognosis However PXA cases having several recurrent patterns with poor prognosis have been reported in recent years and a new concept of anaplastic PXA has been proposed The present case was a 59-year-old woman who presented with tumor bleeding onset and cerebrospinal fluid dissemination The patient had sudden-onset right hemiparesis aphasia and consciousness disturbance and was admitted to a local area hospital After emergency surgery had removed the hematoma postoperative contrast-enhanced CT scan revealed a left temporal tumor A second surgery was therefore performed for initial tumor removal 2 months later Histopathological findings showed that the tumor was typical PXA with strong pleomorphism and xanthomatous changes and contained an ependymoma-like component in the center area However endothelial proliferation and mitosis were more remarkable compared to ordinary PXA The MIB-1 labeling index was 98 high From these findings the histopathological diagnosis was anaplastic PXA The patient underwent surgery to remove recurrent tumors 5 and 16 months later The patient died 36 months after the first onset and CT revealed glioblastoma-like

findings and cerebrospinal fluid dissemination This case report is the first case in which PXA presented with tumor bleeding onset Histopathological findings suggested anaplastic PXA from the first surgical specimens and PXA recurred many times We thus believe that the patient displayed primary anaplastic PXA rather than secondary anaplastic PXA that results in malignant transformation10202016 33unusual giomas

Ganglioglioma

bull WHO grade 1bull Initially described by

Perkins in 1926bull Benign well

differentiatedneuroepithelial tumors

bull Most commonly found intemporal lobe though canoccur anywhere in CNS

bull Commonly found inyoung patients withepilepsy

bull Most commonly notedtumor in temporal lobeepilepsy patients

bull Have been described inbrain steem spinal cordand cerebellum

bull Account for 1 ofprimary brain tumors

10202016 34unusual giomas

bull Male dominencebull Most common features

are seizures headachedizziness ataxiaprogressive weakness

bull Malignanttransformation is rare

bull Histology showsimmature mixture ofabnormal neural andglial elements

bull Macroscopically appearsolid or cystic

bull Cystic are welldelineatedfromsurrounding brain

bull Calcification is common

10202016 35unusual giomas

bull IHC staing shows reactivity for GFAP S-100 andvimentin

bull Neurons show synaptophysin class III betatubulin NFP chromogranin reactivity

10202016 36unusual giomas

Imaging

bull Highly variable imagingcharacters

bull No special typicalimaging criteria

bull Enhancement patternsare highly variable

bull Iso to hypointense onT1WI

bull Heterogenous andhyperintense on T2WI

bull Can contain solid cyticcalcified components

10202016 37unusual giomas

bull FDG PET scan showshypometabolism oreumetabolism in thesepatients

10202016 38unusual giomas

Management And Outcome

bull Gold standard is grosstotal resection

bull Seizure control aftersurgery is as high as80

bull Survival rates are highbull Event free survival of

low grade and highgrade gangiogioma are57 vs 15

bull The optimal surgicalresection depends uponthe eloquent areasinvolved in the vicinityof the tumor

bull The use ofchemotherapy is notwell established

bull Typically they shouldbe allowed for chemoand radiotherapy

10202016 39unusual giomas

bull The use of adjuvant therapy is warranted in thosecases which harbour residual lesions or features ofanaplasia

bull Potential harm to nearby brain areas preclude thejudicious use of radiation

10202016 40unusual giomas

bull Brain Tumor Res Treat 2014 Oct 2(2) 49ndash55

bull Treatment and Outcomes for Gangliogliomas A Single-Center Review of 16 Patients

bull Jye Young Song Jeong Hoon Kim Young Hyun Cho Chang Jin Kim and Eun Jung Lee

bull Ganglioglioma is a rare and slowly growing benign tumor We investigated the outcomes of patients who underwent different combination treatments

bull Methods

bull Between 1998 and 2012 16 patients including 11 men and 5 women with a median age of 125 years (range 25-65 years) were treated for intracranial gangliogliomas at our institution The median follow-up period was 57 years (range 48 days-156 years) Fifteen cases were included in the outcome assessment because one patient was lost to follow-up Complete resection was achieved in 8 (53) patients Six (40) patients underwent incomplete resection with or without adjuvant radiotherapy and one patient with a brainstem tumor underwent only stereotactic biopsy

bull Results

bull Gangliogliomas predominantly affected young (875) male patients and most frequently presented with seizures (64) Of eight patients who underwent complete resection seven did not show recurrence whereas only three of six with incomplete resection showed no recurrence Four patients with recurrence received salvage treatments (two repeat surgeries and two radiosurgeries) A tumor control rate of 93 (1415) was achieved at the last follow-up No recurrence or malignant changes were observed after a median follow-up of 12 and 45 years in four patients who received gamma knife (GK) radiosurgery as adjuvant and salvage treatment

bull Conclusion

bull Complete resection produced the best outcomes and incomplete resection followed by adjuvant or salvage treatments showed favorable outcomes In patients who are not eligible for complete resection because of tumor location or potential neurologic deficits following surgery GK radiosurgery should be considered for the treatment of residual or recurrent tumors

10202016 41unusual giomas

Papillary tumours of pineal

bull WHO grade II AND IIIbull Relatively new edition to WHObull They are equal in both sexesbull Reach medical attention due to headache mental

status changes and altered visionbull Contain eosinophilic cells with distinctive

borders with large pleomorphic nucleibull Positive for cytokeratin synaptophysin stain

variably for GFAP vimentin S-100

10202016 unusual giomas 42

Imaging

bull Generally found to be diffusely enhancinglesion of pineal region

bull Post 3rd ventricle with HCPbull Appear well demarcated from thalamus and

cerebellum

10202016 unusual giomas 43

Mangement

bull Standard treatment is excisionbull Grow slowly and donot transform to

malignant tumorsbull Local recurrence is however possiblebull Radiotherapy appears to provide good control

in local recurrence

10202016 unusual giomas 44

Choroid Glioma Of Third Ventricle

bull WHO Grade IIbull Ist recognised by WHO in

1998bull Arise in the anterior third

ventriclebull Commonly come to

attention after HCPbull Appera to contain in 3rd

ventricle and thought toarise from ventricularsurface

bull Composed of GFAP andvimentin positive cells inmucinous matrix

bull Nuclei are uniform insize and shape

bull Indolent growthbull May show EGF receptor

positivitybull Some suggets these tumor

arise from the lminaterminalis andventricular tanycytes

10202016 45unusual giomas

Imaging

bull Commonly fill 3rd

ventriclebull Diffusely enhance with

contrastbull Appear to infiltrate the

thalamusbull Large tumors may be

difficult to differentiatefrom suprasellar masses

10202016 46unusual giomas

10202016 47unusual giomas

bull Surgical management is limited by locationbull Access through the dialted ventricle and lamina

terminalis may be challangingbull Subtotal resection is thus commonbull Role of adjuvant radiotherapy is unknown

10202016 48unusual giomas

bull Acta Neurochir (Wien) 2008 Nov150(11)1183-91 doi 101007s00701-008-0014-6 Epub

2008 Oct 21

bull Chordoid glioma of the third ventricle

bull Vanhauwaert DJ1 Clement F Van Dorpe J Deruytter MJKGROUND

bull Chordoid glioma is a rare tumour (World Health Organisation grade II) originating from the third ventricle

with both glial and chordoid features It was first described by Brat in 1998 Since there is no detailed

information available on the outcome after surgery and adjuvant treatment we reviewed the literature

bull METHODS

bull A literature search through PUBMED revealed 50 cases of chordoid glioma Most reports

were found in pathology journals Information on the postoperative course was sometimes

very limited We reviewed the available literature and studied in detail the presenting

symptoms mortality and postoperative complications in relation to the extent of resective

surgery as well as the importance of adjuvant treatment

bull CONCLUSIONS

bull Mortality in the immediate postoperative period is 32 and is higher after gross total

resection as compared to subtotal resection Non-fatal postoperative complications are

hypothalamic disorders and mental alterations Gross total resection is the treatment of

choice since no recurrence has been reported after macroscopically complete resection

but this is often difficult because of the location and adherence to the hypothalamus The

role of postoperative radiotherapy is uncertain There is some indication that radiosurgery

with or without conventional irradiation is superior to conventional radiation alone Planned

subtotal resection followed by stereotactic radiosurgery can be a safe and effective

alternative in a patient in whom gross total resection is considered to be too risky

There is no report on the use of chemotherapy in the treatment of chordoid gliomas More

information about the optimal treatment strategy is needed and more reports are also needed10202016 49unusual giomas

bull Neurologist 2011 Jan17(1)52bull Chordoid glioma a case report and literature reviewbull Liu WP1 Cheng JX Yi XC Zhen HN Fei Z Li Q Zhang Xbull chordoid glioma is a rare tumor (World Health Organization grade II) with both

glial and chordoid features often located in the suprasellar region and anterior third ventricle It was first described by Brat in 1998 Because there is no detailed information available from the clinical perspective we reviewed the literature

bull METHODS

bull a literature search through PUBMED and CNKI revealed 64 cases of chordoid glioma Information on the clinical course was very limited We reviewed the literature and studied the pathologic and imaging features postoperative mortality and morbidity in relation to surgical extension and approaches and the importance of adjuvant treatment

bull CONCLUSIONS bull mortality in the immediate postoperative period is 28 and postoperative

morbidity is 60 which are statistically higher after gross total resection as compared with subtotal resection Translamina terminalis approach is considered to be the best approach The current study cannot document that patients have longer survival and higher quality of life after gross total resection than subtotal resection The role of postoperative radiotherapy is uncertain and there is no report on the use of chemotherapy More information about the optimal treatment strategy is needed

10202016 50unusual giomas

Conclusion

bull They are identified by their unique morphologicalfeatures

bull Many of these have indolent course and identified byepilepsy

bull Potentail diagnosis should be considered in youngpatients with seizures and cortically based lesions

bull Correct diagnosis requires experiencedneuropathologisrt

bull As more experienced is gained more knowledgetowards better understanding shall provide betteroutcomes in these patients

10202016 unusual giomas 51

10202016 52unusual giomas

Content supportndash youmann neurosurgery textbook

Management

bull Do not require interventionunless bolck the foramen ofmonroe

bull Primary treatment issurgical total resection

bull Subtotal resection may beadequate in case totalresection is hazardous

bull Tumor rarely becomesaggressive

bull Tarnscortical transcallosalapproach

bull If surgery not possible ordenied by pt thenmanagement of HCP byshunt

bull Role of chemotherapy hasshown promises

10202016 7unusual giomas

Everolimus for Subependymal Giant-Cell Astrocytomas in Tuberous SclerosisDarcy A Krueger MD PhD Marguerite M Care MD Katherine Holland MD PhD KarenAgricola FNP Cynthia Tudor PNP Prajakta Mangeshkar MS Kimberly A Wilson MSAnna Byars PhD Tarek Sahmoud MD PhD and David Neal Franz MDN Engl J Med 2010 3631801-181

bull Backgroundbull Neurosurgical resection is the standard treatment for subependymal giant-cell astrocytomas in patients with the tuberous

sclerosis complex An alternative may be the use of everolimus which inhibits the mammalian target of rapamycin a protein regulated by gene products involved in the tuberous sclerosis complex

bull Methodsbull Patients 3 years of age or older with serial growth of subependymal giant-cell astrocytomas were eligible for this open-label

study The primary efficacy end point was the change in volume of subependymal giant-cell astrocytomas between baseline and 6 months We gave everolimus orally at a dose of 30 mg per square meter of body-surface area to achieve a trough concentration of 5 to 15 ng per milliliter

bull Resultsbull We enrolled 28 patients Everolimus therapy was associated with a clinically meaningful reduction in volume of the

primary subependymal giant-cell astrocytoma as assessed on independent central review (Plt0001 for baseline vs 6 months) with a reduction of at least 30 in 21 patients (75) and at least 50 in 9 patients (32) Marked reductions were seen within 3 months and were sustained There were no new lesions worsening hydrocephalus evidence of increased intracranial pressure or necessity for surgical resection or other therapy for subependymal giant-cell astrocytoma Of the 16 patients for whom 24-hour video electroencephalography data were available seizure frequency for the 6-month study period (vs the previous 6-month period) decreased in 9 did not change in 6 and increased in 1 (median change minus1 seizure P=002) The mean (plusmnSD) score on the validated Quality-of-Life in Childhood Epilepsy questionnaire (on which scores can range from 0 to 100 with higher scores indicating a better quality of life) was improved at 3 months (634plusmn124) and 6 months (621plusmn142) over the baseline score (578plusmn140) Single cases of grade 3 treatment-related sinusitis pneumonia viral bronchitis tooth infection stomatitis and leukopenia were reported

bull Conclusionsbull Everolimus therapy was associated with marked reduction in the volume of subependymal giant-cell

astrocytomas and seizure frequency and may be a potential alternative to neurosurgical resection in some cases though long-term studies are needed

10202016 8unusual giomas

Angiocentric glioma

bull Described by WHO in2005

bull Initially included withastroblastoma andchoroid glioma

bull Mean age at diagnosis is17 years

bull Pts present with longhistory of seizures

bull Microscopically show themonomorphous bipolarcells associated withnormal vessels of thecortex and white matter

bull Tumor cells are spindleshaped

bull Mitoses are rarebull Positive for S-100 GFAP

vimentin

10202016 9unusual giomas

Imaging

bull CT images show it as ahypodense lesion at thecortex

bull MR shows a mass withstalk towards theventricular surfacemdashpathognomic

bull Hypointense to iso onT1WI

bull Hyperintense on T2WIbull Post contrast

enhancement is sparse

10202016 10unusual giomas

10202016 11unusual giomas

Management and outcome

bull Follow an indolent coursebull Rare progressionbull Surgical cure obtained by total resectionbull Aggressive forms are exceedingly rare

10202016 12unusual giomas

bull Perm J 2013 Winter17(1)e100-2 doi 107812TPP12-060

bull The treatment of angiocentric glioma case report and literature review

bull Alexandru D1 Haghighi B Muhonen MG

bull Abstract

bull Angiocentric glioma is a recently described tumor recognized since 2007

by the World Health Organization Classification of Tumours of the Central

Nervous System We present the only case of angiocentric glioma at our

institution in the last 15 years and review the literature in an attempt to

establish prognostic parameters Our search revealed only 27 cases of

angiocentric glioma in the literature The most common presenting

symptom of angiocentric glioma was seizures Gross total resection of

the lesion was curative without need for radiation or chemotherapy

10202016 13unusual giomas

Astroblastoma

bull WHO grade 1bull Initially described by Bailey

and Bucy in 1930bull Has been a matter of debate

for similar features asanaplastic astrocytomaglioblastoma andgemistocytic astrocytomas

bull Commonly found in 1st

three decades of lifebull Female

preponderance(111)

bull Affects the hemispheresinvolving cortex subcortical andperiventricular regions

bull No infratentorail tumorhave been reported

bull Pts present with features ofraised ICP cortical deficitsseizures personalitychanges

10202016 14unusual giomas

bull Greatly variable naturalhistory

bull Have a slow andindolent course

bull Rarely show anaggressive outcome

bull Almost always definedby their grossappearance

bull Cut surface revealhomogenous soft pinkgrey substances

bull Cystic areas arefrequently encountered

bull The clusters of cellsform pseudorossette

10202016 15unusual giomas

bull Richly supplied byblood vessels

bull Nuclei of tumor cellsare away from bloodvessels and send fineprocesse to the vesselwall

bull High number of mitoticfigures can be found

bull Cellular atypia isfrequently noted

bull On IHC tumor cellsstain positive for GFAP

bull Positivity for vimentinNSE S-1OO EMA

bull These are positive foranti leu 7 antibodiesspecific for cells ofepithelial origin

bull Central necrosis can befound

10202016 16unusual giomas

Imaging

bull On CT the appearnacesvaries from poorlydefined hypodensetumor to welldemarcated and highlyenhancing masses

10202016 17unusual giomas

bull On MR image tumorsare hypointense towhite matter on T1

bull Hyperintense on T2bull Hyperintense on FLAIRbull Enhancement is seen

around the cystic centre

10202016 18unusual giomas

Management And Prognosis

bull Surgical resection is main treatmentbull Gross total resection can be easily achieved as

lesions are mostly in the cortical areasbull Therapeutic value is of radiation and

chemotherapy is uncertain as these are raretumors

bull Prediction of outcome is difficult as these remainindolent for a long time and may rapidlytransform to glioblastoma resulting in fataloutcomes

10202016 19unusual giomas

Pilomyxoid astrocytoma

bull Recently described tumorsbull Similarities with pilocytic

astrocytomasbull Until recently these were

grouped with PAbull These involve entire

neuraxisbull Mean age at preentation is

18 monthsbull Most are found in

hypothalamic ndash chiasmicregion

bull Pts present with features ofraised ICP

bull RAISED HEADCIRCUMFERENCE may beonly subtle sign in infants

bull Histologically consists ofmyxoid matrix with cells inloose fibrillary and myxoidbackground

bull Tumor cells formperivascular rosettes

10202016 20unusual giomas

bull Preferred sites includebull (1) optic nerve (lsquooptic

nerve gliomarsquo)bull (2) optic

chiasmhypothalamusbull (3) thalamus and basal

gangliabull (4) cerebral hemispherebull (5) cerebellum (lsquocerebellar

astrocytomarsquo) andbull (6) brainstem (dorsal

exophytic brainstemglioma)

bull Genetically chracterized bythe presence of NF1mutations BRAFduplications and theabsence of IDH1 mutation

10202016 21unusual giomas

Imaging

bull The huge chiasmatic-hypothalamic tumor revealslow signal intensity on aT1-weighted image (A)

bull Bright signal intensity on aT2-weighted image (B)

bull Gadolinium-enhancedbrain MR images showheterogeneously strongenhancement (C and D)

bull Leptomeningealdisseminations at thecervical spine levels

10202016 22unusual giomas

bull NO standard careprotocols have beendefined

bull As these are newlydefined and raretumors

bull Surgical resection isfavoured if locationfavours

bull Mean progression freesurvival is 25 months

bull Mean overall survival is60 months

bull Reduced survival isbecause of inability toachieve gross resection

10202016 23unusual giomas

bull The usual indications foradjuvant treatment(chemotherapy orradiation therapy)include tumor recurrenceafter initial completeresection or symptomatictumors

bull In addition adjuvanttreatment is generallyindicated for tumors withgrowth on follow-upimaging even in theabsence of symptoms

bull The role of chemotherapyis in evolution

bull Chemotherapy may beimplemented for the treatment ofinoperable or partially resectedgliomas

bull The decision to use postoperativeadjuvant therapy also depends ona lesions perceived malignanttendencies as the potentialbenefits of radiation therapy andchemotherapy must be weighedagainst their morbidity

bull The decision to use postoperativeadjuvant therapy also depends ona lesions perceived malignanttendencies as the potentialbenefits of radiation therapy andchemotherapy must be weighedagainst their morbidity

10202016 24unusual giomas

Pleomorphic xanthoastrocytoma

bull First described by Kepsand colleuges in 1979

bull Represents lt1 of allastrocytomas

bull May be found alongentire neuraxis mostlysupratentorial( temporallobe)

bull Cerebellum and soinalcord are the other sites

bull Pts present in 2nd and 3rd

decadesbull Median age at 14 yearsbull Both sexes are affected

equallybull 70-80 pts present with

seizures and headachefocal neuro deficits

bull Rarely tumor mayhemmorrhage

10202016 25unusual giomas

bull Supratentorail are mostoften cortical and meningesbased

bull Chronic seizuresbull Grossely these are firm

tumors variable in colorrelatively avascular

bull Typically invade the piaarachnoid space

bull 13 pts have involvementof all the three meninges

bull Cyst fluid is xanthochromic

bull Very specific microscpoiccriteria for diagnosis are

1 Pleomorphic andxanthomatous astrocytes

2 Perivascular lymphocytes3 Non infilterating histology4 Reticulin stainingbull Pleomorphism can be as

vast as glioblastoma orsarcomatous tumors

10202016 26unusual giomas

bull IHC Staining revealsspindle cells and roundpleomorphic cellspositive for S-100 andGFAP

bull Staining forsynaptophysin and NSEmay be also present

bull Some workers havereported them to be asubtype of ganglioglioma

bull Association between theNeurofibromatosis Sturge Weber Syndrome

bull Loss of chr 9 and gain ofchromosome 7 have beenfound

10202016 27unusual giomas

Imaging

bull CTbull PXAs are typically hypo

or isodense and may bewell or poorlydemarcated usually withno or little surroundingoedema

bull Calcification is rarebull Due to its superficial

location it may causescalloping of theoverlying bone

10202016 28unusual giomas

bull MRIbull T1

ndash solid component iso tohypointense cf grey matter

ndash cystic component low signalndash leptomeningeal involvement

seen in over 70 of casesbull T1 C+ (Gd)

ndash solid component usuallyenhances vividly

bull T2ndash solid component iso to

hyperintense cf grey matterndash cystic component high signalndash on T2 FLAIR sequence cystic

areas show hyperintensityrelative to CSF due to higherprotein contents

ndash little surrounding vasogenicoedema

10202016 29unusual giomas

Management amp Outcome

bull Quite rare tumorsbull Surgical excision appears to

be best favoured modalitybull Actuarial survival trates are

91 at 5 years and 82 at10 years

bull Median survival 18 years

bull Total resection should beattempted in every case asfar as possible

bull Resection of cyst lining isnot indicated

bull Data regarding the use ofadjuvant therapy at thistime are limited to few casereports only

bull Pts who had incompleteresection high mitoticindex necrosis should bereserved for strict vigilanceand CEMRI on follow ups

10202016 30unusual giomas

bull At progression repeatresection be attempted

bull Proression should betaken as aggressivetumor type

bull Malignanttransformation maynecessitate adjuvanttherapy

10202016 31unusual giomas

bull Br J Neurosurg 2013 Dec27(6)759-64 doi 103109026886972013776666 Epub 2013 Mar 20

bull Pleomorphic xanthoastrocytoma long-term results of surgical treatment and analysis of prognosticfactors

bull Gallo P1 Cecchi PC Locatelli F Rizzo P Ghimenton C Gerosa M Pinna G

bull BACKGROUND

bull Pleomorphic Xanthoastrocytoma (PXA) is a rare brain tumour most commonly affecting children andyoung adults To date only few data regarding the long-term follow-up of these patients after surgery are

available The aim of this study is to describe our single-institution experience in the surgicalmanagement of this particular glioma over a period of over 18 years

bull METHODS

bull We performed a retrospective review of all cases of PXA (40 patients) operated upon at theDepartment of Neurosurgery of Verona Italy between 1990 and 2008 The impact of clinical radiologicalsurgical and histological factors on overall survival (OS) and progression-free survival (PFS) was analysed bymeans of univariate and multivariate models

bull FINDINGS

bull We achieved a gross total resection (GTR) in 65 of patients Histological diagnosis was of grade II in 80anaplastic features were present in the remaining 20 Adjuvant treatment radiotherapy or chemo-

radiotherapy was administered in 40 of the cases Median follow-up was 74 months OS at 5- and10 years was 7632 and 6824 respectively PFS at 5- and 10 years was 71 and 58respectively In the multivariate model histological grade extent of resection and age at diagnosis (le 30years vs gt 30 years) were the only independent prognostic factors for both OS and PFS

bull CONCLUSIONS

bull Our retrospective long-term study confirms the relatively favourable prognosis associatedwith PXA Young patients with a low-grade tumour (WHO grade II) who underwent GTR carrythe longest OS and PFS

10202016 32unusual giomas

bull Brain Tumor Pathol 2006 Apr23(1)55-63

bull A case of anaplastic pleomorphic xanthoastrocytoma presenting with tumor bleeding and cerebrospinal fluid dissemination

bull Asano K1 Miyamoto S Kubo O Kikkukawa T Yagihashi A Ohkuma H

bull Pleomorphic xanthoastrocytoma (PXA) has been considered an astrocytic tumorwith a relatively favorable prognosis However PXA cases having several recurrent patterns with poor prognosis have been reported in recent years and a new concept of anaplastic PXA has been proposed The present case was a 59-year-old woman who presented with tumor bleeding onset and cerebrospinal fluid dissemination The patient had sudden-onset right hemiparesis aphasia and consciousness disturbance and was admitted to a local area hospital After emergency surgery had removed the hematoma postoperative contrast-enhanced CT scan revealed a left temporal tumor A second surgery was therefore performed for initial tumor removal 2 months later Histopathological findings showed that the tumor was typical PXA with strong pleomorphism and xanthomatous changes and contained an ependymoma-like component in the center area However endothelial proliferation and mitosis were more remarkable compared to ordinary PXA The MIB-1 labeling index was 98 high From these findings the histopathological diagnosis was anaplastic PXA The patient underwent surgery to remove recurrent tumors 5 and 16 months later The patient died 36 months after the first onset and CT revealed glioblastoma-like

findings and cerebrospinal fluid dissemination This case report is the first case in which PXA presented with tumor bleeding onset Histopathological findings suggested anaplastic PXA from the first surgical specimens and PXA recurred many times We thus believe that the patient displayed primary anaplastic PXA rather than secondary anaplastic PXA that results in malignant transformation10202016 33unusual giomas

Ganglioglioma

bull WHO grade 1bull Initially described by

Perkins in 1926bull Benign well

differentiatedneuroepithelial tumors

bull Most commonly found intemporal lobe though canoccur anywhere in CNS

bull Commonly found inyoung patients withepilepsy

bull Most commonly notedtumor in temporal lobeepilepsy patients

bull Have been described inbrain steem spinal cordand cerebellum

bull Account for 1 ofprimary brain tumors

10202016 34unusual giomas

bull Male dominencebull Most common features

are seizures headachedizziness ataxiaprogressive weakness

bull Malignanttransformation is rare

bull Histology showsimmature mixture ofabnormal neural andglial elements

bull Macroscopically appearsolid or cystic

bull Cystic are welldelineatedfromsurrounding brain

bull Calcification is common

10202016 35unusual giomas

bull IHC staing shows reactivity for GFAP S-100 andvimentin

bull Neurons show synaptophysin class III betatubulin NFP chromogranin reactivity

10202016 36unusual giomas

Imaging

bull Highly variable imagingcharacters

bull No special typicalimaging criteria

bull Enhancement patternsare highly variable

bull Iso to hypointense onT1WI

bull Heterogenous andhyperintense on T2WI

bull Can contain solid cyticcalcified components

10202016 37unusual giomas

bull FDG PET scan showshypometabolism oreumetabolism in thesepatients

10202016 38unusual giomas

Management And Outcome

bull Gold standard is grosstotal resection

bull Seizure control aftersurgery is as high as80

bull Survival rates are highbull Event free survival of

low grade and highgrade gangiogioma are57 vs 15

bull The optimal surgicalresection depends uponthe eloquent areasinvolved in the vicinityof the tumor

bull The use ofchemotherapy is notwell established

bull Typically they shouldbe allowed for chemoand radiotherapy

10202016 39unusual giomas

bull The use of adjuvant therapy is warranted in thosecases which harbour residual lesions or features ofanaplasia

bull Potential harm to nearby brain areas preclude thejudicious use of radiation

10202016 40unusual giomas

bull Brain Tumor Res Treat 2014 Oct 2(2) 49ndash55

bull Treatment and Outcomes for Gangliogliomas A Single-Center Review of 16 Patients

bull Jye Young Song Jeong Hoon Kim Young Hyun Cho Chang Jin Kim and Eun Jung Lee

bull Ganglioglioma is a rare and slowly growing benign tumor We investigated the outcomes of patients who underwent different combination treatments

bull Methods

bull Between 1998 and 2012 16 patients including 11 men and 5 women with a median age of 125 years (range 25-65 years) were treated for intracranial gangliogliomas at our institution The median follow-up period was 57 years (range 48 days-156 years) Fifteen cases were included in the outcome assessment because one patient was lost to follow-up Complete resection was achieved in 8 (53) patients Six (40) patients underwent incomplete resection with or without adjuvant radiotherapy and one patient with a brainstem tumor underwent only stereotactic biopsy

bull Results

bull Gangliogliomas predominantly affected young (875) male patients and most frequently presented with seizures (64) Of eight patients who underwent complete resection seven did not show recurrence whereas only three of six with incomplete resection showed no recurrence Four patients with recurrence received salvage treatments (two repeat surgeries and two radiosurgeries) A tumor control rate of 93 (1415) was achieved at the last follow-up No recurrence or malignant changes were observed after a median follow-up of 12 and 45 years in four patients who received gamma knife (GK) radiosurgery as adjuvant and salvage treatment

bull Conclusion

bull Complete resection produced the best outcomes and incomplete resection followed by adjuvant or salvage treatments showed favorable outcomes In patients who are not eligible for complete resection because of tumor location or potential neurologic deficits following surgery GK radiosurgery should be considered for the treatment of residual or recurrent tumors

10202016 41unusual giomas

Papillary tumours of pineal

bull WHO grade II AND IIIbull Relatively new edition to WHObull They are equal in both sexesbull Reach medical attention due to headache mental

status changes and altered visionbull Contain eosinophilic cells with distinctive

borders with large pleomorphic nucleibull Positive for cytokeratin synaptophysin stain

variably for GFAP vimentin S-100

10202016 unusual giomas 42

Imaging

bull Generally found to be diffusely enhancinglesion of pineal region

bull Post 3rd ventricle with HCPbull Appear well demarcated from thalamus and

cerebellum

10202016 unusual giomas 43

Mangement

bull Standard treatment is excisionbull Grow slowly and donot transform to

malignant tumorsbull Local recurrence is however possiblebull Radiotherapy appears to provide good control

in local recurrence

10202016 unusual giomas 44

Choroid Glioma Of Third Ventricle

bull WHO Grade IIbull Ist recognised by WHO in

1998bull Arise in the anterior third

ventriclebull Commonly come to

attention after HCPbull Appera to contain in 3rd

ventricle and thought toarise from ventricularsurface

bull Composed of GFAP andvimentin positive cells inmucinous matrix

bull Nuclei are uniform insize and shape

bull Indolent growthbull May show EGF receptor

positivitybull Some suggets these tumor

arise from the lminaterminalis andventricular tanycytes

10202016 45unusual giomas

Imaging

bull Commonly fill 3rd

ventriclebull Diffusely enhance with

contrastbull Appear to infiltrate the

thalamusbull Large tumors may be

difficult to differentiatefrom suprasellar masses

10202016 46unusual giomas

10202016 47unusual giomas

bull Surgical management is limited by locationbull Access through the dialted ventricle and lamina

terminalis may be challangingbull Subtotal resection is thus commonbull Role of adjuvant radiotherapy is unknown

10202016 48unusual giomas

bull Acta Neurochir (Wien) 2008 Nov150(11)1183-91 doi 101007s00701-008-0014-6 Epub

2008 Oct 21

bull Chordoid glioma of the third ventricle

bull Vanhauwaert DJ1 Clement F Van Dorpe J Deruytter MJKGROUND

bull Chordoid glioma is a rare tumour (World Health Organisation grade II) originating from the third ventricle

with both glial and chordoid features It was first described by Brat in 1998 Since there is no detailed

information available on the outcome after surgery and adjuvant treatment we reviewed the literature

bull METHODS

bull A literature search through PUBMED revealed 50 cases of chordoid glioma Most reports

were found in pathology journals Information on the postoperative course was sometimes

very limited We reviewed the available literature and studied in detail the presenting

symptoms mortality and postoperative complications in relation to the extent of resective

surgery as well as the importance of adjuvant treatment

bull CONCLUSIONS

bull Mortality in the immediate postoperative period is 32 and is higher after gross total

resection as compared to subtotal resection Non-fatal postoperative complications are

hypothalamic disorders and mental alterations Gross total resection is the treatment of

choice since no recurrence has been reported after macroscopically complete resection

but this is often difficult because of the location and adherence to the hypothalamus The

role of postoperative radiotherapy is uncertain There is some indication that radiosurgery

with or without conventional irradiation is superior to conventional radiation alone Planned

subtotal resection followed by stereotactic radiosurgery can be a safe and effective

alternative in a patient in whom gross total resection is considered to be too risky

There is no report on the use of chemotherapy in the treatment of chordoid gliomas More

information about the optimal treatment strategy is needed and more reports are also needed10202016 49unusual giomas

bull Neurologist 2011 Jan17(1)52bull Chordoid glioma a case report and literature reviewbull Liu WP1 Cheng JX Yi XC Zhen HN Fei Z Li Q Zhang Xbull chordoid glioma is a rare tumor (World Health Organization grade II) with both

glial and chordoid features often located in the suprasellar region and anterior third ventricle It was first described by Brat in 1998 Because there is no detailed information available from the clinical perspective we reviewed the literature

bull METHODS

bull a literature search through PUBMED and CNKI revealed 64 cases of chordoid glioma Information on the clinical course was very limited We reviewed the literature and studied the pathologic and imaging features postoperative mortality and morbidity in relation to surgical extension and approaches and the importance of adjuvant treatment

bull CONCLUSIONS bull mortality in the immediate postoperative period is 28 and postoperative

morbidity is 60 which are statistically higher after gross total resection as compared with subtotal resection Translamina terminalis approach is considered to be the best approach The current study cannot document that patients have longer survival and higher quality of life after gross total resection than subtotal resection The role of postoperative radiotherapy is uncertain and there is no report on the use of chemotherapy More information about the optimal treatment strategy is needed

10202016 50unusual giomas

Conclusion

bull They are identified by their unique morphologicalfeatures

bull Many of these have indolent course and identified byepilepsy

bull Potentail diagnosis should be considered in youngpatients with seizures and cortically based lesions

bull Correct diagnosis requires experiencedneuropathologisrt

bull As more experienced is gained more knowledgetowards better understanding shall provide betteroutcomes in these patients

10202016 unusual giomas 51

10202016 52unusual giomas

Content supportndash youmann neurosurgery textbook

Everolimus for Subependymal Giant-Cell Astrocytomas in Tuberous SclerosisDarcy A Krueger MD PhD Marguerite M Care MD Katherine Holland MD PhD KarenAgricola FNP Cynthia Tudor PNP Prajakta Mangeshkar MS Kimberly A Wilson MSAnna Byars PhD Tarek Sahmoud MD PhD and David Neal Franz MDN Engl J Med 2010 3631801-181

bull Backgroundbull Neurosurgical resection is the standard treatment for subependymal giant-cell astrocytomas in patients with the tuberous

sclerosis complex An alternative may be the use of everolimus which inhibits the mammalian target of rapamycin a protein regulated by gene products involved in the tuberous sclerosis complex

bull Methodsbull Patients 3 years of age or older with serial growth of subependymal giant-cell astrocytomas were eligible for this open-label

study The primary efficacy end point was the change in volume of subependymal giant-cell astrocytomas between baseline and 6 months We gave everolimus orally at a dose of 30 mg per square meter of body-surface area to achieve a trough concentration of 5 to 15 ng per milliliter

bull Resultsbull We enrolled 28 patients Everolimus therapy was associated with a clinically meaningful reduction in volume of the

primary subependymal giant-cell astrocytoma as assessed on independent central review (Plt0001 for baseline vs 6 months) with a reduction of at least 30 in 21 patients (75) and at least 50 in 9 patients (32) Marked reductions were seen within 3 months and were sustained There were no new lesions worsening hydrocephalus evidence of increased intracranial pressure or necessity for surgical resection or other therapy for subependymal giant-cell astrocytoma Of the 16 patients for whom 24-hour video electroencephalography data were available seizure frequency for the 6-month study period (vs the previous 6-month period) decreased in 9 did not change in 6 and increased in 1 (median change minus1 seizure P=002) The mean (plusmnSD) score on the validated Quality-of-Life in Childhood Epilepsy questionnaire (on which scores can range from 0 to 100 with higher scores indicating a better quality of life) was improved at 3 months (634plusmn124) and 6 months (621plusmn142) over the baseline score (578plusmn140) Single cases of grade 3 treatment-related sinusitis pneumonia viral bronchitis tooth infection stomatitis and leukopenia were reported

bull Conclusionsbull Everolimus therapy was associated with marked reduction in the volume of subependymal giant-cell

astrocytomas and seizure frequency and may be a potential alternative to neurosurgical resection in some cases though long-term studies are needed

10202016 8unusual giomas

Angiocentric glioma

bull Described by WHO in2005

bull Initially included withastroblastoma andchoroid glioma

bull Mean age at diagnosis is17 years

bull Pts present with longhistory of seizures

bull Microscopically show themonomorphous bipolarcells associated withnormal vessels of thecortex and white matter

bull Tumor cells are spindleshaped

bull Mitoses are rarebull Positive for S-100 GFAP

vimentin

10202016 9unusual giomas

Imaging

bull CT images show it as ahypodense lesion at thecortex

bull MR shows a mass withstalk towards theventricular surfacemdashpathognomic

bull Hypointense to iso onT1WI

bull Hyperintense on T2WIbull Post contrast

enhancement is sparse

10202016 10unusual giomas

10202016 11unusual giomas

Management and outcome

bull Follow an indolent coursebull Rare progressionbull Surgical cure obtained by total resectionbull Aggressive forms are exceedingly rare

10202016 12unusual giomas

bull Perm J 2013 Winter17(1)e100-2 doi 107812TPP12-060

bull The treatment of angiocentric glioma case report and literature review

bull Alexandru D1 Haghighi B Muhonen MG

bull Abstract

bull Angiocentric glioma is a recently described tumor recognized since 2007

by the World Health Organization Classification of Tumours of the Central

Nervous System We present the only case of angiocentric glioma at our

institution in the last 15 years and review the literature in an attempt to

establish prognostic parameters Our search revealed only 27 cases of

angiocentric glioma in the literature The most common presenting

symptom of angiocentric glioma was seizures Gross total resection of

the lesion was curative without need for radiation or chemotherapy

10202016 13unusual giomas

Astroblastoma

bull WHO grade 1bull Initially described by Bailey

and Bucy in 1930bull Has been a matter of debate

for similar features asanaplastic astrocytomaglioblastoma andgemistocytic astrocytomas

bull Commonly found in 1st

three decades of lifebull Female

preponderance(111)

bull Affects the hemispheresinvolving cortex subcortical andperiventricular regions

bull No infratentorail tumorhave been reported

bull Pts present with features ofraised ICP cortical deficitsseizures personalitychanges

10202016 14unusual giomas

bull Greatly variable naturalhistory

bull Have a slow andindolent course

bull Rarely show anaggressive outcome

bull Almost always definedby their grossappearance

bull Cut surface revealhomogenous soft pinkgrey substances

bull Cystic areas arefrequently encountered

bull The clusters of cellsform pseudorossette

10202016 15unusual giomas

bull Richly supplied byblood vessels

bull Nuclei of tumor cellsare away from bloodvessels and send fineprocesse to the vesselwall

bull High number of mitoticfigures can be found

bull Cellular atypia isfrequently noted

bull On IHC tumor cellsstain positive for GFAP

bull Positivity for vimentinNSE S-1OO EMA

bull These are positive foranti leu 7 antibodiesspecific for cells ofepithelial origin

bull Central necrosis can befound

10202016 16unusual giomas

Imaging

bull On CT the appearnacesvaries from poorlydefined hypodensetumor to welldemarcated and highlyenhancing masses

10202016 17unusual giomas

bull On MR image tumorsare hypointense towhite matter on T1

bull Hyperintense on T2bull Hyperintense on FLAIRbull Enhancement is seen

around the cystic centre

10202016 18unusual giomas

Management And Prognosis

bull Surgical resection is main treatmentbull Gross total resection can be easily achieved as

lesions are mostly in the cortical areasbull Therapeutic value is of radiation and

chemotherapy is uncertain as these are raretumors

bull Prediction of outcome is difficult as these remainindolent for a long time and may rapidlytransform to glioblastoma resulting in fataloutcomes

10202016 19unusual giomas

Pilomyxoid astrocytoma

bull Recently described tumorsbull Similarities with pilocytic

astrocytomasbull Until recently these were

grouped with PAbull These involve entire

neuraxisbull Mean age at preentation is

18 monthsbull Most are found in

hypothalamic ndash chiasmicregion

bull Pts present with features ofraised ICP

bull RAISED HEADCIRCUMFERENCE may beonly subtle sign in infants

bull Histologically consists ofmyxoid matrix with cells inloose fibrillary and myxoidbackground

bull Tumor cells formperivascular rosettes

10202016 20unusual giomas

bull Preferred sites includebull (1) optic nerve (lsquooptic

nerve gliomarsquo)bull (2) optic

chiasmhypothalamusbull (3) thalamus and basal

gangliabull (4) cerebral hemispherebull (5) cerebellum (lsquocerebellar

astrocytomarsquo) andbull (6) brainstem (dorsal

exophytic brainstemglioma)

bull Genetically chracterized bythe presence of NF1mutations BRAFduplications and theabsence of IDH1 mutation

10202016 21unusual giomas

Imaging

bull The huge chiasmatic-hypothalamic tumor revealslow signal intensity on aT1-weighted image (A)

bull Bright signal intensity on aT2-weighted image (B)

bull Gadolinium-enhancedbrain MR images showheterogeneously strongenhancement (C and D)

bull Leptomeningealdisseminations at thecervical spine levels

10202016 22unusual giomas

bull NO standard careprotocols have beendefined

bull As these are newlydefined and raretumors

bull Surgical resection isfavoured if locationfavours

bull Mean progression freesurvival is 25 months

bull Mean overall survival is60 months

bull Reduced survival isbecause of inability toachieve gross resection

10202016 23unusual giomas

bull The usual indications foradjuvant treatment(chemotherapy orradiation therapy)include tumor recurrenceafter initial completeresection or symptomatictumors

bull In addition adjuvanttreatment is generallyindicated for tumors withgrowth on follow-upimaging even in theabsence of symptoms

bull The role of chemotherapyis in evolution

bull Chemotherapy may beimplemented for the treatment ofinoperable or partially resectedgliomas

bull The decision to use postoperativeadjuvant therapy also depends ona lesions perceived malignanttendencies as the potentialbenefits of radiation therapy andchemotherapy must be weighedagainst their morbidity

bull The decision to use postoperativeadjuvant therapy also depends ona lesions perceived malignanttendencies as the potentialbenefits of radiation therapy andchemotherapy must be weighedagainst their morbidity

10202016 24unusual giomas

Pleomorphic xanthoastrocytoma

bull First described by Kepsand colleuges in 1979

bull Represents lt1 of allastrocytomas

bull May be found alongentire neuraxis mostlysupratentorial( temporallobe)

bull Cerebellum and soinalcord are the other sites

bull Pts present in 2nd and 3rd

decadesbull Median age at 14 yearsbull Both sexes are affected

equallybull 70-80 pts present with

seizures and headachefocal neuro deficits

bull Rarely tumor mayhemmorrhage

10202016 25unusual giomas

bull Supratentorail are mostoften cortical and meningesbased

bull Chronic seizuresbull Grossely these are firm

tumors variable in colorrelatively avascular

bull Typically invade the piaarachnoid space

bull 13 pts have involvementof all the three meninges

bull Cyst fluid is xanthochromic

bull Very specific microscpoiccriteria for diagnosis are

1 Pleomorphic andxanthomatous astrocytes

2 Perivascular lymphocytes3 Non infilterating histology4 Reticulin stainingbull Pleomorphism can be as

vast as glioblastoma orsarcomatous tumors

10202016 26unusual giomas

bull IHC Staining revealsspindle cells and roundpleomorphic cellspositive for S-100 andGFAP

bull Staining forsynaptophysin and NSEmay be also present

bull Some workers havereported them to be asubtype of ganglioglioma

bull Association between theNeurofibromatosis Sturge Weber Syndrome

bull Loss of chr 9 and gain ofchromosome 7 have beenfound

10202016 27unusual giomas

Imaging

bull CTbull PXAs are typically hypo

or isodense and may bewell or poorlydemarcated usually withno or little surroundingoedema

bull Calcification is rarebull Due to its superficial

location it may causescalloping of theoverlying bone

10202016 28unusual giomas

bull MRIbull T1

ndash solid component iso tohypointense cf grey matter

ndash cystic component low signalndash leptomeningeal involvement

seen in over 70 of casesbull T1 C+ (Gd)

ndash solid component usuallyenhances vividly

bull T2ndash solid component iso to

hyperintense cf grey matterndash cystic component high signalndash on T2 FLAIR sequence cystic

areas show hyperintensityrelative to CSF due to higherprotein contents

ndash little surrounding vasogenicoedema

10202016 29unusual giomas

Management amp Outcome

bull Quite rare tumorsbull Surgical excision appears to

be best favoured modalitybull Actuarial survival trates are

91 at 5 years and 82 at10 years

bull Median survival 18 years

bull Total resection should beattempted in every case asfar as possible

bull Resection of cyst lining isnot indicated

bull Data regarding the use ofadjuvant therapy at thistime are limited to few casereports only

bull Pts who had incompleteresection high mitoticindex necrosis should bereserved for strict vigilanceand CEMRI on follow ups

10202016 30unusual giomas

bull At progression repeatresection be attempted

bull Proression should betaken as aggressivetumor type

bull Malignanttransformation maynecessitate adjuvanttherapy

10202016 31unusual giomas

bull Br J Neurosurg 2013 Dec27(6)759-64 doi 103109026886972013776666 Epub 2013 Mar 20

bull Pleomorphic xanthoastrocytoma long-term results of surgical treatment and analysis of prognosticfactors

bull Gallo P1 Cecchi PC Locatelli F Rizzo P Ghimenton C Gerosa M Pinna G

bull BACKGROUND

bull Pleomorphic Xanthoastrocytoma (PXA) is a rare brain tumour most commonly affecting children andyoung adults To date only few data regarding the long-term follow-up of these patients after surgery are

available The aim of this study is to describe our single-institution experience in the surgicalmanagement of this particular glioma over a period of over 18 years

bull METHODS

bull We performed a retrospective review of all cases of PXA (40 patients) operated upon at theDepartment of Neurosurgery of Verona Italy between 1990 and 2008 The impact of clinical radiologicalsurgical and histological factors on overall survival (OS) and progression-free survival (PFS) was analysed bymeans of univariate and multivariate models

bull FINDINGS

bull We achieved a gross total resection (GTR) in 65 of patients Histological diagnosis was of grade II in 80anaplastic features were present in the remaining 20 Adjuvant treatment radiotherapy or chemo-

radiotherapy was administered in 40 of the cases Median follow-up was 74 months OS at 5- and10 years was 7632 and 6824 respectively PFS at 5- and 10 years was 71 and 58respectively In the multivariate model histological grade extent of resection and age at diagnosis (le 30years vs gt 30 years) were the only independent prognostic factors for both OS and PFS

bull CONCLUSIONS

bull Our retrospective long-term study confirms the relatively favourable prognosis associatedwith PXA Young patients with a low-grade tumour (WHO grade II) who underwent GTR carrythe longest OS and PFS

10202016 32unusual giomas

bull Brain Tumor Pathol 2006 Apr23(1)55-63

bull A case of anaplastic pleomorphic xanthoastrocytoma presenting with tumor bleeding and cerebrospinal fluid dissemination

bull Asano K1 Miyamoto S Kubo O Kikkukawa T Yagihashi A Ohkuma H

bull Pleomorphic xanthoastrocytoma (PXA) has been considered an astrocytic tumorwith a relatively favorable prognosis However PXA cases having several recurrent patterns with poor prognosis have been reported in recent years and a new concept of anaplastic PXA has been proposed The present case was a 59-year-old woman who presented with tumor bleeding onset and cerebrospinal fluid dissemination The patient had sudden-onset right hemiparesis aphasia and consciousness disturbance and was admitted to a local area hospital After emergency surgery had removed the hematoma postoperative contrast-enhanced CT scan revealed a left temporal tumor A second surgery was therefore performed for initial tumor removal 2 months later Histopathological findings showed that the tumor was typical PXA with strong pleomorphism and xanthomatous changes and contained an ependymoma-like component in the center area However endothelial proliferation and mitosis were more remarkable compared to ordinary PXA The MIB-1 labeling index was 98 high From these findings the histopathological diagnosis was anaplastic PXA The patient underwent surgery to remove recurrent tumors 5 and 16 months later The patient died 36 months after the first onset and CT revealed glioblastoma-like

findings and cerebrospinal fluid dissemination This case report is the first case in which PXA presented with tumor bleeding onset Histopathological findings suggested anaplastic PXA from the first surgical specimens and PXA recurred many times We thus believe that the patient displayed primary anaplastic PXA rather than secondary anaplastic PXA that results in malignant transformation10202016 33unusual giomas

Ganglioglioma

bull WHO grade 1bull Initially described by

Perkins in 1926bull Benign well

differentiatedneuroepithelial tumors

bull Most commonly found intemporal lobe though canoccur anywhere in CNS

bull Commonly found inyoung patients withepilepsy

bull Most commonly notedtumor in temporal lobeepilepsy patients

bull Have been described inbrain steem spinal cordand cerebellum

bull Account for 1 ofprimary brain tumors

10202016 34unusual giomas

bull Male dominencebull Most common features

are seizures headachedizziness ataxiaprogressive weakness

bull Malignanttransformation is rare

bull Histology showsimmature mixture ofabnormal neural andglial elements

bull Macroscopically appearsolid or cystic

bull Cystic are welldelineatedfromsurrounding brain

bull Calcification is common

10202016 35unusual giomas

bull IHC staing shows reactivity for GFAP S-100 andvimentin

bull Neurons show synaptophysin class III betatubulin NFP chromogranin reactivity

10202016 36unusual giomas

Imaging

bull Highly variable imagingcharacters

bull No special typicalimaging criteria

bull Enhancement patternsare highly variable

bull Iso to hypointense onT1WI

bull Heterogenous andhyperintense on T2WI

bull Can contain solid cyticcalcified components

10202016 37unusual giomas

bull FDG PET scan showshypometabolism oreumetabolism in thesepatients

10202016 38unusual giomas

Management And Outcome

bull Gold standard is grosstotal resection

bull Seizure control aftersurgery is as high as80

bull Survival rates are highbull Event free survival of

low grade and highgrade gangiogioma are57 vs 15

bull The optimal surgicalresection depends uponthe eloquent areasinvolved in the vicinityof the tumor

bull The use ofchemotherapy is notwell established

bull Typically they shouldbe allowed for chemoand radiotherapy

10202016 39unusual giomas

bull The use of adjuvant therapy is warranted in thosecases which harbour residual lesions or features ofanaplasia

bull Potential harm to nearby brain areas preclude thejudicious use of radiation

10202016 40unusual giomas

bull Brain Tumor Res Treat 2014 Oct 2(2) 49ndash55

bull Treatment and Outcomes for Gangliogliomas A Single-Center Review of 16 Patients

bull Jye Young Song Jeong Hoon Kim Young Hyun Cho Chang Jin Kim and Eun Jung Lee

bull Ganglioglioma is a rare and slowly growing benign tumor We investigated the outcomes of patients who underwent different combination treatments

bull Methods

bull Between 1998 and 2012 16 patients including 11 men and 5 women with a median age of 125 years (range 25-65 years) were treated for intracranial gangliogliomas at our institution The median follow-up period was 57 years (range 48 days-156 years) Fifteen cases were included in the outcome assessment because one patient was lost to follow-up Complete resection was achieved in 8 (53) patients Six (40) patients underwent incomplete resection with or without adjuvant radiotherapy and one patient with a brainstem tumor underwent only stereotactic biopsy

bull Results

bull Gangliogliomas predominantly affected young (875) male patients and most frequently presented with seizures (64) Of eight patients who underwent complete resection seven did not show recurrence whereas only three of six with incomplete resection showed no recurrence Four patients with recurrence received salvage treatments (two repeat surgeries and two radiosurgeries) A tumor control rate of 93 (1415) was achieved at the last follow-up No recurrence or malignant changes were observed after a median follow-up of 12 and 45 years in four patients who received gamma knife (GK) radiosurgery as adjuvant and salvage treatment

bull Conclusion

bull Complete resection produced the best outcomes and incomplete resection followed by adjuvant or salvage treatments showed favorable outcomes In patients who are not eligible for complete resection because of tumor location or potential neurologic deficits following surgery GK radiosurgery should be considered for the treatment of residual or recurrent tumors

10202016 41unusual giomas

Papillary tumours of pineal

bull WHO grade II AND IIIbull Relatively new edition to WHObull They are equal in both sexesbull Reach medical attention due to headache mental

status changes and altered visionbull Contain eosinophilic cells with distinctive

borders with large pleomorphic nucleibull Positive for cytokeratin synaptophysin stain

variably for GFAP vimentin S-100

10202016 unusual giomas 42

Imaging

bull Generally found to be diffusely enhancinglesion of pineal region

bull Post 3rd ventricle with HCPbull Appear well demarcated from thalamus and

cerebellum

10202016 unusual giomas 43

Mangement

bull Standard treatment is excisionbull Grow slowly and donot transform to

malignant tumorsbull Local recurrence is however possiblebull Radiotherapy appears to provide good control

in local recurrence

10202016 unusual giomas 44

Choroid Glioma Of Third Ventricle

bull WHO Grade IIbull Ist recognised by WHO in

1998bull Arise in the anterior third

ventriclebull Commonly come to

attention after HCPbull Appera to contain in 3rd

ventricle and thought toarise from ventricularsurface

bull Composed of GFAP andvimentin positive cells inmucinous matrix

bull Nuclei are uniform insize and shape

bull Indolent growthbull May show EGF receptor

positivitybull Some suggets these tumor

arise from the lminaterminalis andventricular tanycytes

10202016 45unusual giomas

Imaging

bull Commonly fill 3rd

ventriclebull Diffusely enhance with

contrastbull Appear to infiltrate the

thalamusbull Large tumors may be

difficult to differentiatefrom suprasellar masses

10202016 46unusual giomas

10202016 47unusual giomas

bull Surgical management is limited by locationbull Access through the dialted ventricle and lamina

terminalis may be challangingbull Subtotal resection is thus commonbull Role of adjuvant radiotherapy is unknown

10202016 48unusual giomas

bull Acta Neurochir (Wien) 2008 Nov150(11)1183-91 doi 101007s00701-008-0014-6 Epub

2008 Oct 21

bull Chordoid glioma of the third ventricle

bull Vanhauwaert DJ1 Clement F Van Dorpe J Deruytter MJKGROUND

bull Chordoid glioma is a rare tumour (World Health Organisation grade II) originating from the third ventricle

with both glial and chordoid features It was first described by Brat in 1998 Since there is no detailed

information available on the outcome after surgery and adjuvant treatment we reviewed the literature

bull METHODS

bull A literature search through PUBMED revealed 50 cases of chordoid glioma Most reports

were found in pathology journals Information on the postoperative course was sometimes

very limited We reviewed the available literature and studied in detail the presenting

symptoms mortality and postoperative complications in relation to the extent of resective

surgery as well as the importance of adjuvant treatment

bull CONCLUSIONS

bull Mortality in the immediate postoperative period is 32 and is higher after gross total

resection as compared to subtotal resection Non-fatal postoperative complications are

hypothalamic disorders and mental alterations Gross total resection is the treatment of

choice since no recurrence has been reported after macroscopically complete resection

but this is often difficult because of the location and adherence to the hypothalamus The

role of postoperative radiotherapy is uncertain There is some indication that radiosurgery

with or without conventional irradiation is superior to conventional radiation alone Planned

subtotal resection followed by stereotactic radiosurgery can be a safe and effective

alternative in a patient in whom gross total resection is considered to be too risky

There is no report on the use of chemotherapy in the treatment of chordoid gliomas More

information about the optimal treatment strategy is needed and more reports are also needed10202016 49unusual giomas

bull Neurologist 2011 Jan17(1)52bull Chordoid glioma a case report and literature reviewbull Liu WP1 Cheng JX Yi XC Zhen HN Fei Z Li Q Zhang Xbull chordoid glioma is a rare tumor (World Health Organization grade II) with both

glial and chordoid features often located in the suprasellar region and anterior third ventricle It was first described by Brat in 1998 Because there is no detailed information available from the clinical perspective we reviewed the literature

bull METHODS

bull a literature search through PUBMED and CNKI revealed 64 cases of chordoid glioma Information on the clinical course was very limited We reviewed the literature and studied the pathologic and imaging features postoperative mortality and morbidity in relation to surgical extension and approaches and the importance of adjuvant treatment

bull CONCLUSIONS bull mortality in the immediate postoperative period is 28 and postoperative

morbidity is 60 which are statistically higher after gross total resection as compared with subtotal resection Translamina terminalis approach is considered to be the best approach The current study cannot document that patients have longer survival and higher quality of life after gross total resection than subtotal resection The role of postoperative radiotherapy is uncertain and there is no report on the use of chemotherapy More information about the optimal treatment strategy is needed

10202016 50unusual giomas

Conclusion

bull They are identified by their unique morphologicalfeatures

bull Many of these have indolent course and identified byepilepsy

bull Potentail diagnosis should be considered in youngpatients with seizures and cortically based lesions

bull Correct diagnosis requires experiencedneuropathologisrt

bull As more experienced is gained more knowledgetowards better understanding shall provide betteroutcomes in these patients

10202016 unusual giomas 51

10202016 52unusual giomas

Content supportndash youmann neurosurgery textbook

Angiocentric glioma

bull Described by WHO in2005

bull Initially included withastroblastoma andchoroid glioma

bull Mean age at diagnosis is17 years

bull Pts present with longhistory of seizures

bull Microscopically show themonomorphous bipolarcells associated withnormal vessels of thecortex and white matter

bull Tumor cells are spindleshaped

bull Mitoses are rarebull Positive for S-100 GFAP

vimentin

10202016 9unusual giomas

Imaging

bull CT images show it as ahypodense lesion at thecortex

bull MR shows a mass withstalk towards theventricular surfacemdashpathognomic

bull Hypointense to iso onT1WI

bull Hyperintense on T2WIbull Post contrast

enhancement is sparse

10202016 10unusual giomas

10202016 11unusual giomas

Management and outcome

bull Follow an indolent coursebull Rare progressionbull Surgical cure obtained by total resectionbull Aggressive forms are exceedingly rare

10202016 12unusual giomas

bull Perm J 2013 Winter17(1)e100-2 doi 107812TPP12-060

bull The treatment of angiocentric glioma case report and literature review

bull Alexandru D1 Haghighi B Muhonen MG

bull Abstract

bull Angiocentric glioma is a recently described tumor recognized since 2007

by the World Health Organization Classification of Tumours of the Central

Nervous System We present the only case of angiocentric glioma at our

institution in the last 15 years and review the literature in an attempt to

establish prognostic parameters Our search revealed only 27 cases of

angiocentric glioma in the literature The most common presenting

symptom of angiocentric glioma was seizures Gross total resection of

the lesion was curative without need for radiation or chemotherapy

10202016 13unusual giomas

Astroblastoma

bull WHO grade 1bull Initially described by Bailey

and Bucy in 1930bull Has been a matter of debate

for similar features asanaplastic astrocytomaglioblastoma andgemistocytic astrocytomas

bull Commonly found in 1st

three decades of lifebull Female

preponderance(111)

bull Affects the hemispheresinvolving cortex subcortical andperiventricular regions

bull No infratentorail tumorhave been reported

bull Pts present with features ofraised ICP cortical deficitsseizures personalitychanges

10202016 14unusual giomas

bull Greatly variable naturalhistory

bull Have a slow andindolent course

bull Rarely show anaggressive outcome

bull Almost always definedby their grossappearance

bull Cut surface revealhomogenous soft pinkgrey substances

bull Cystic areas arefrequently encountered

bull The clusters of cellsform pseudorossette

10202016 15unusual giomas

bull Richly supplied byblood vessels

bull Nuclei of tumor cellsare away from bloodvessels and send fineprocesse to the vesselwall

bull High number of mitoticfigures can be found

bull Cellular atypia isfrequently noted

bull On IHC tumor cellsstain positive for GFAP

bull Positivity for vimentinNSE S-1OO EMA

bull These are positive foranti leu 7 antibodiesspecific for cells ofepithelial origin

bull Central necrosis can befound

10202016 16unusual giomas

Imaging

bull On CT the appearnacesvaries from poorlydefined hypodensetumor to welldemarcated and highlyenhancing masses

10202016 17unusual giomas

bull On MR image tumorsare hypointense towhite matter on T1

bull Hyperintense on T2bull Hyperintense on FLAIRbull Enhancement is seen

around the cystic centre

10202016 18unusual giomas

Management And Prognosis

bull Surgical resection is main treatmentbull Gross total resection can be easily achieved as

lesions are mostly in the cortical areasbull Therapeutic value is of radiation and

chemotherapy is uncertain as these are raretumors

bull Prediction of outcome is difficult as these remainindolent for a long time and may rapidlytransform to glioblastoma resulting in fataloutcomes

10202016 19unusual giomas

Pilomyxoid astrocytoma

bull Recently described tumorsbull Similarities with pilocytic

astrocytomasbull Until recently these were

grouped with PAbull These involve entire

neuraxisbull Mean age at preentation is

18 monthsbull Most are found in

hypothalamic ndash chiasmicregion

bull Pts present with features ofraised ICP

bull RAISED HEADCIRCUMFERENCE may beonly subtle sign in infants

bull Histologically consists ofmyxoid matrix with cells inloose fibrillary and myxoidbackground

bull Tumor cells formperivascular rosettes

10202016 20unusual giomas

bull Preferred sites includebull (1) optic nerve (lsquooptic

nerve gliomarsquo)bull (2) optic

chiasmhypothalamusbull (3) thalamus and basal

gangliabull (4) cerebral hemispherebull (5) cerebellum (lsquocerebellar

astrocytomarsquo) andbull (6) brainstem (dorsal

exophytic brainstemglioma)

bull Genetically chracterized bythe presence of NF1mutations BRAFduplications and theabsence of IDH1 mutation

10202016 21unusual giomas

Imaging

bull The huge chiasmatic-hypothalamic tumor revealslow signal intensity on aT1-weighted image (A)

bull Bright signal intensity on aT2-weighted image (B)

bull Gadolinium-enhancedbrain MR images showheterogeneously strongenhancement (C and D)

bull Leptomeningealdisseminations at thecervical spine levels

10202016 22unusual giomas

bull NO standard careprotocols have beendefined

bull As these are newlydefined and raretumors

bull Surgical resection isfavoured if locationfavours

bull Mean progression freesurvival is 25 months

bull Mean overall survival is60 months

bull Reduced survival isbecause of inability toachieve gross resection

10202016 23unusual giomas

bull The usual indications foradjuvant treatment(chemotherapy orradiation therapy)include tumor recurrenceafter initial completeresection or symptomatictumors

bull In addition adjuvanttreatment is generallyindicated for tumors withgrowth on follow-upimaging even in theabsence of symptoms

bull The role of chemotherapyis in evolution

bull Chemotherapy may beimplemented for the treatment ofinoperable or partially resectedgliomas

bull The decision to use postoperativeadjuvant therapy also depends ona lesions perceived malignanttendencies as the potentialbenefits of radiation therapy andchemotherapy must be weighedagainst their morbidity

bull The decision to use postoperativeadjuvant therapy also depends ona lesions perceived malignanttendencies as the potentialbenefits of radiation therapy andchemotherapy must be weighedagainst their morbidity

10202016 24unusual giomas

Pleomorphic xanthoastrocytoma

bull First described by Kepsand colleuges in 1979

bull Represents lt1 of allastrocytomas

bull May be found alongentire neuraxis mostlysupratentorial( temporallobe)

bull Cerebellum and soinalcord are the other sites

bull Pts present in 2nd and 3rd

decadesbull Median age at 14 yearsbull Both sexes are affected

equallybull 70-80 pts present with

seizures and headachefocal neuro deficits

bull Rarely tumor mayhemmorrhage

10202016 25unusual giomas

bull Supratentorail are mostoften cortical and meningesbased

bull Chronic seizuresbull Grossely these are firm

tumors variable in colorrelatively avascular

bull Typically invade the piaarachnoid space

bull 13 pts have involvementof all the three meninges

bull Cyst fluid is xanthochromic

bull Very specific microscpoiccriteria for diagnosis are

1 Pleomorphic andxanthomatous astrocytes

2 Perivascular lymphocytes3 Non infilterating histology4 Reticulin stainingbull Pleomorphism can be as

vast as glioblastoma orsarcomatous tumors

10202016 26unusual giomas

bull IHC Staining revealsspindle cells and roundpleomorphic cellspositive for S-100 andGFAP

bull Staining forsynaptophysin and NSEmay be also present

bull Some workers havereported them to be asubtype of ganglioglioma

bull Association between theNeurofibromatosis Sturge Weber Syndrome

bull Loss of chr 9 and gain ofchromosome 7 have beenfound

10202016 27unusual giomas

Imaging

bull CTbull PXAs are typically hypo

or isodense and may bewell or poorlydemarcated usually withno or little surroundingoedema

bull Calcification is rarebull Due to its superficial

location it may causescalloping of theoverlying bone

10202016 28unusual giomas

bull MRIbull T1

ndash solid component iso tohypointense cf grey matter

ndash cystic component low signalndash leptomeningeal involvement

seen in over 70 of casesbull T1 C+ (Gd)

ndash solid component usuallyenhances vividly

bull T2ndash solid component iso to

hyperintense cf grey matterndash cystic component high signalndash on T2 FLAIR sequence cystic

areas show hyperintensityrelative to CSF due to higherprotein contents

ndash little surrounding vasogenicoedema

10202016 29unusual giomas

Management amp Outcome

bull Quite rare tumorsbull Surgical excision appears to

be best favoured modalitybull Actuarial survival trates are

91 at 5 years and 82 at10 years

bull Median survival 18 years

bull Total resection should beattempted in every case asfar as possible

bull Resection of cyst lining isnot indicated

bull Data regarding the use ofadjuvant therapy at thistime are limited to few casereports only

bull Pts who had incompleteresection high mitoticindex necrosis should bereserved for strict vigilanceand CEMRI on follow ups

10202016 30unusual giomas

bull At progression repeatresection be attempted

bull Proression should betaken as aggressivetumor type

bull Malignanttransformation maynecessitate adjuvanttherapy

10202016 31unusual giomas

bull Br J Neurosurg 2013 Dec27(6)759-64 doi 103109026886972013776666 Epub 2013 Mar 20

bull Pleomorphic xanthoastrocytoma long-term results of surgical treatment and analysis of prognosticfactors

bull Gallo P1 Cecchi PC Locatelli F Rizzo P Ghimenton C Gerosa M Pinna G

bull BACKGROUND

bull Pleomorphic Xanthoastrocytoma (PXA) is a rare brain tumour most commonly affecting children andyoung adults To date only few data regarding the long-term follow-up of these patients after surgery are

available The aim of this study is to describe our single-institution experience in the surgicalmanagement of this particular glioma over a period of over 18 years

bull METHODS

bull We performed a retrospective review of all cases of PXA (40 patients) operated upon at theDepartment of Neurosurgery of Verona Italy between 1990 and 2008 The impact of clinical radiologicalsurgical and histological factors on overall survival (OS) and progression-free survival (PFS) was analysed bymeans of univariate and multivariate models

bull FINDINGS

bull We achieved a gross total resection (GTR) in 65 of patients Histological diagnosis was of grade II in 80anaplastic features were present in the remaining 20 Adjuvant treatment radiotherapy or chemo-

radiotherapy was administered in 40 of the cases Median follow-up was 74 months OS at 5- and10 years was 7632 and 6824 respectively PFS at 5- and 10 years was 71 and 58respectively In the multivariate model histological grade extent of resection and age at diagnosis (le 30years vs gt 30 years) were the only independent prognostic factors for both OS and PFS

bull CONCLUSIONS

bull Our retrospective long-term study confirms the relatively favourable prognosis associatedwith PXA Young patients with a low-grade tumour (WHO grade II) who underwent GTR carrythe longest OS and PFS

10202016 32unusual giomas

bull Brain Tumor Pathol 2006 Apr23(1)55-63

bull A case of anaplastic pleomorphic xanthoastrocytoma presenting with tumor bleeding and cerebrospinal fluid dissemination

bull Asano K1 Miyamoto S Kubo O Kikkukawa T Yagihashi A Ohkuma H

bull Pleomorphic xanthoastrocytoma (PXA) has been considered an astrocytic tumorwith a relatively favorable prognosis However PXA cases having several recurrent patterns with poor prognosis have been reported in recent years and a new concept of anaplastic PXA has been proposed The present case was a 59-year-old woman who presented with tumor bleeding onset and cerebrospinal fluid dissemination The patient had sudden-onset right hemiparesis aphasia and consciousness disturbance and was admitted to a local area hospital After emergency surgery had removed the hematoma postoperative contrast-enhanced CT scan revealed a left temporal tumor A second surgery was therefore performed for initial tumor removal 2 months later Histopathological findings showed that the tumor was typical PXA with strong pleomorphism and xanthomatous changes and contained an ependymoma-like component in the center area However endothelial proliferation and mitosis were more remarkable compared to ordinary PXA The MIB-1 labeling index was 98 high From these findings the histopathological diagnosis was anaplastic PXA The patient underwent surgery to remove recurrent tumors 5 and 16 months later The patient died 36 months after the first onset and CT revealed glioblastoma-like

findings and cerebrospinal fluid dissemination This case report is the first case in which PXA presented with tumor bleeding onset Histopathological findings suggested anaplastic PXA from the first surgical specimens and PXA recurred many times We thus believe that the patient displayed primary anaplastic PXA rather than secondary anaplastic PXA that results in malignant transformation10202016 33unusual giomas

Ganglioglioma

bull WHO grade 1bull Initially described by

Perkins in 1926bull Benign well

differentiatedneuroepithelial tumors

bull Most commonly found intemporal lobe though canoccur anywhere in CNS

bull Commonly found inyoung patients withepilepsy

bull Most commonly notedtumor in temporal lobeepilepsy patients

bull Have been described inbrain steem spinal cordand cerebellum

bull Account for 1 ofprimary brain tumors

10202016 34unusual giomas

bull Male dominencebull Most common features

are seizures headachedizziness ataxiaprogressive weakness

bull Malignanttransformation is rare

bull Histology showsimmature mixture ofabnormal neural andglial elements

bull Macroscopically appearsolid or cystic

bull Cystic are welldelineatedfromsurrounding brain

bull Calcification is common

10202016 35unusual giomas

bull IHC staing shows reactivity for GFAP S-100 andvimentin

bull Neurons show synaptophysin class III betatubulin NFP chromogranin reactivity

10202016 36unusual giomas

Imaging

bull Highly variable imagingcharacters

bull No special typicalimaging criteria

bull Enhancement patternsare highly variable

bull Iso to hypointense onT1WI

bull Heterogenous andhyperintense on T2WI

bull Can contain solid cyticcalcified components

10202016 37unusual giomas

bull FDG PET scan showshypometabolism oreumetabolism in thesepatients

10202016 38unusual giomas

Management And Outcome

bull Gold standard is grosstotal resection

bull Seizure control aftersurgery is as high as80

bull Survival rates are highbull Event free survival of

low grade and highgrade gangiogioma are57 vs 15

bull The optimal surgicalresection depends uponthe eloquent areasinvolved in the vicinityof the tumor

bull The use ofchemotherapy is notwell established

bull Typically they shouldbe allowed for chemoand radiotherapy

10202016 39unusual giomas

bull The use of adjuvant therapy is warranted in thosecases which harbour residual lesions or features ofanaplasia

bull Potential harm to nearby brain areas preclude thejudicious use of radiation

10202016 40unusual giomas

bull Brain Tumor Res Treat 2014 Oct 2(2) 49ndash55

bull Treatment and Outcomes for Gangliogliomas A Single-Center Review of 16 Patients

bull Jye Young Song Jeong Hoon Kim Young Hyun Cho Chang Jin Kim and Eun Jung Lee

bull Ganglioglioma is a rare and slowly growing benign tumor We investigated the outcomes of patients who underwent different combination treatments

bull Methods

bull Between 1998 and 2012 16 patients including 11 men and 5 women with a median age of 125 years (range 25-65 years) were treated for intracranial gangliogliomas at our institution The median follow-up period was 57 years (range 48 days-156 years) Fifteen cases were included in the outcome assessment because one patient was lost to follow-up Complete resection was achieved in 8 (53) patients Six (40) patients underwent incomplete resection with or without adjuvant radiotherapy and one patient with a brainstem tumor underwent only stereotactic biopsy

bull Results

bull Gangliogliomas predominantly affected young (875) male patients and most frequently presented with seizures (64) Of eight patients who underwent complete resection seven did not show recurrence whereas only three of six with incomplete resection showed no recurrence Four patients with recurrence received salvage treatments (two repeat surgeries and two radiosurgeries) A tumor control rate of 93 (1415) was achieved at the last follow-up No recurrence or malignant changes were observed after a median follow-up of 12 and 45 years in four patients who received gamma knife (GK) radiosurgery as adjuvant and salvage treatment

bull Conclusion

bull Complete resection produced the best outcomes and incomplete resection followed by adjuvant or salvage treatments showed favorable outcomes In patients who are not eligible for complete resection because of tumor location or potential neurologic deficits following surgery GK radiosurgery should be considered for the treatment of residual or recurrent tumors

10202016 41unusual giomas

Papillary tumours of pineal

bull WHO grade II AND IIIbull Relatively new edition to WHObull They are equal in both sexesbull Reach medical attention due to headache mental

status changes and altered visionbull Contain eosinophilic cells with distinctive

borders with large pleomorphic nucleibull Positive for cytokeratin synaptophysin stain

variably for GFAP vimentin S-100

10202016 unusual giomas 42

Imaging

bull Generally found to be diffusely enhancinglesion of pineal region

bull Post 3rd ventricle with HCPbull Appear well demarcated from thalamus and

cerebellum

10202016 unusual giomas 43

Mangement

bull Standard treatment is excisionbull Grow slowly and donot transform to

malignant tumorsbull Local recurrence is however possiblebull Radiotherapy appears to provide good control

in local recurrence

10202016 unusual giomas 44

Choroid Glioma Of Third Ventricle

bull WHO Grade IIbull Ist recognised by WHO in

1998bull Arise in the anterior third

ventriclebull Commonly come to

attention after HCPbull Appera to contain in 3rd

ventricle and thought toarise from ventricularsurface

bull Composed of GFAP andvimentin positive cells inmucinous matrix

bull Nuclei are uniform insize and shape

bull Indolent growthbull May show EGF receptor

positivitybull Some suggets these tumor

arise from the lminaterminalis andventricular tanycytes

10202016 45unusual giomas

Imaging

bull Commonly fill 3rd

ventriclebull Diffusely enhance with

contrastbull Appear to infiltrate the

thalamusbull Large tumors may be

difficult to differentiatefrom suprasellar masses

10202016 46unusual giomas

10202016 47unusual giomas

bull Surgical management is limited by locationbull Access through the dialted ventricle and lamina

terminalis may be challangingbull Subtotal resection is thus commonbull Role of adjuvant radiotherapy is unknown

10202016 48unusual giomas

bull Acta Neurochir (Wien) 2008 Nov150(11)1183-91 doi 101007s00701-008-0014-6 Epub

2008 Oct 21

bull Chordoid glioma of the third ventricle

bull Vanhauwaert DJ1 Clement F Van Dorpe J Deruytter MJKGROUND

bull Chordoid glioma is a rare tumour (World Health Organisation grade II) originating from the third ventricle

with both glial and chordoid features It was first described by Brat in 1998 Since there is no detailed

information available on the outcome after surgery and adjuvant treatment we reviewed the literature

bull METHODS

bull A literature search through PUBMED revealed 50 cases of chordoid glioma Most reports

were found in pathology journals Information on the postoperative course was sometimes

very limited We reviewed the available literature and studied in detail the presenting

symptoms mortality and postoperative complications in relation to the extent of resective

surgery as well as the importance of adjuvant treatment

bull CONCLUSIONS

bull Mortality in the immediate postoperative period is 32 and is higher after gross total

resection as compared to subtotal resection Non-fatal postoperative complications are

hypothalamic disorders and mental alterations Gross total resection is the treatment of

choice since no recurrence has been reported after macroscopically complete resection

but this is often difficult because of the location and adherence to the hypothalamus The

role of postoperative radiotherapy is uncertain There is some indication that radiosurgery

with or without conventional irradiation is superior to conventional radiation alone Planned

subtotal resection followed by stereotactic radiosurgery can be a safe and effective

alternative in a patient in whom gross total resection is considered to be too risky

There is no report on the use of chemotherapy in the treatment of chordoid gliomas More

information about the optimal treatment strategy is needed and more reports are also needed10202016 49unusual giomas

bull Neurologist 2011 Jan17(1)52bull Chordoid glioma a case report and literature reviewbull Liu WP1 Cheng JX Yi XC Zhen HN Fei Z Li Q Zhang Xbull chordoid glioma is a rare tumor (World Health Organization grade II) with both

glial and chordoid features often located in the suprasellar region and anterior third ventricle It was first described by Brat in 1998 Because there is no detailed information available from the clinical perspective we reviewed the literature

bull METHODS

bull a literature search through PUBMED and CNKI revealed 64 cases of chordoid glioma Information on the clinical course was very limited We reviewed the literature and studied the pathologic and imaging features postoperative mortality and morbidity in relation to surgical extension and approaches and the importance of adjuvant treatment

bull CONCLUSIONS bull mortality in the immediate postoperative period is 28 and postoperative

morbidity is 60 which are statistically higher after gross total resection as compared with subtotal resection Translamina terminalis approach is considered to be the best approach The current study cannot document that patients have longer survival and higher quality of life after gross total resection than subtotal resection The role of postoperative radiotherapy is uncertain and there is no report on the use of chemotherapy More information about the optimal treatment strategy is needed

10202016 50unusual giomas

Conclusion

bull They are identified by their unique morphologicalfeatures

bull Many of these have indolent course and identified byepilepsy

bull Potentail diagnosis should be considered in youngpatients with seizures and cortically based lesions

bull Correct diagnosis requires experiencedneuropathologisrt

bull As more experienced is gained more knowledgetowards better understanding shall provide betteroutcomes in these patients

10202016 unusual giomas 51

10202016 52unusual giomas

Content supportndash youmann neurosurgery textbook

Imaging

bull CT images show it as ahypodense lesion at thecortex

bull MR shows a mass withstalk towards theventricular surfacemdashpathognomic

bull Hypointense to iso onT1WI

bull Hyperintense on T2WIbull Post contrast

enhancement is sparse

10202016 10unusual giomas

10202016 11unusual giomas

Management and outcome

bull Follow an indolent coursebull Rare progressionbull Surgical cure obtained by total resectionbull Aggressive forms are exceedingly rare

10202016 12unusual giomas

bull Perm J 2013 Winter17(1)e100-2 doi 107812TPP12-060

bull The treatment of angiocentric glioma case report and literature review

bull Alexandru D1 Haghighi B Muhonen MG

bull Abstract

bull Angiocentric glioma is a recently described tumor recognized since 2007

by the World Health Organization Classification of Tumours of the Central

Nervous System We present the only case of angiocentric glioma at our

institution in the last 15 years and review the literature in an attempt to

establish prognostic parameters Our search revealed only 27 cases of

angiocentric glioma in the literature The most common presenting

symptom of angiocentric glioma was seizures Gross total resection of

the lesion was curative without need for radiation or chemotherapy

10202016 13unusual giomas

Astroblastoma

bull WHO grade 1bull Initially described by Bailey

and Bucy in 1930bull Has been a matter of debate

for similar features asanaplastic astrocytomaglioblastoma andgemistocytic astrocytomas

bull Commonly found in 1st

three decades of lifebull Female

preponderance(111)

bull Affects the hemispheresinvolving cortex subcortical andperiventricular regions

bull No infratentorail tumorhave been reported

bull Pts present with features ofraised ICP cortical deficitsseizures personalitychanges

10202016 14unusual giomas

bull Greatly variable naturalhistory

bull Have a slow andindolent course

bull Rarely show anaggressive outcome

bull Almost always definedby their grossappearance

bull Cut surface revealhomogenous soft pinkgrey substances

bull Cystic areas arefrequently encountered

bull The clusters of cellsform pseudorossette

10202016 15unusual giomas

bull Richly supplied byblood vessels

bull Nuclei of tumor cellsare away from bloodvessels and send fineprocesse to the vesselwall

bull High number of mitoticfigures can be found

bull Cellular atypia isfrequently noted

bull On IHC tumor cellsstain positive for GFAP

bull Positivity for vimentinNSE S-1OO EMA

bull These are positive foranti leu 7 antibodiesspecific for cells ofepithelial origin

bull Central necrosis can befound

10202016 16unusual giomas

Imaging

bull On CT the appearnacesvaries from poorlydefined hypodensetumor to welldemarcated and highlyenhancing masses

10202016 17unusual giomas

bull On MR image tumorsare hypointense towhite matter on T1

bull Hyperintense on T2bull Hyperintense on FLAIRbull Enhancement is seen

around the cystic centre

10202016 18unusual giomas

Management And Prognosis

bull Surgical resection is main treatmentbull Gross total resection can be easily achieved as

lesions are mostly in the cortical areasbull Therapeutic value is of radiation and

chemotherapy is uncertain as these are raretumors

bull Prediction of outcome is difficult as these remainindolent for a long time and may rapidlytransform to glioblastoma resulting in fataloutcomes

10202016 19unusual giomas

Pilomyxoid astrocytoma

bull Recently described tumorsbull Similarities with pilocytic

astrocytomasbull Until recently these were

grouped with PAbull These involve entire

neuraxisbull Mean age at preentation is

18 monthsbull Most are found in

hypothalamic ndash chiasmicregion

bull Pts present with features ofraised ICP

bull RAISED HEADCIRCUMFERENCE may beonly subtle sign in infants

bull Histologically consists ofmyxoid matrix with cells inloose fibrillary and myxoidbackground

bull Tumor cells formperivascular rosettes

10202016 20unusual giomas

bull Preferred sites includebull (1) optic nerve (lsquooptic

nerve gliomarsquo)bull (2) optic

chiasmhypothalamusbull (3) thalamus and basal

gangliabull (4) cerebral hemispherebull (5) cerebellum (lsquocerebellar

astrocytomarsquo) andbull (6) brainstem (dorsal

exophytic brainstemglioma)

bull Genetically chracterized bythe presence of NF1mutations BRAFduplications and theabsence of IDH1 mutation

10202016 21unusual giomas

Imaging

bull The huge chiasmatic-hypothalamic tumor revealslow signal intensity on aT1-weighted image (A)

bull Bright signal intensity on aT2-weighted image (B)

bull Gadolinium-enhancedbrain MR images showheterogeneously strongenhancement (C and D)

bull Leptomeningealdisseminations at thecervical spine levels

10202016 22unusual giomas

bull NO standard careprotocols have beendefined

bull As these are newlydefined and raretumors

bull Surgical resection isfavoured if locationfavours

bull Mean progression freesurvival is 25 months

bull Mean overall survival is60 months

bull Reduced survival isbecause of inability toachieve gross resection

10202016 23unusual giomas

bull The usual indications foradjuvant treatment(chemotherapy orradiation therapy)include tumor recurrenceafter initial completeresection or symptomatictumors

bull In addition adjuvanttreatment is generallyindicated for tumors withgrowth on follow-upimaging even in theabsence of symptoms

bull The role of chemotherapyis in evolution

bull Chemotherapy may beimplemented for the treatment ofinoperable or partially resectedgliomas

bull The decision to use postoperativeadjuvant therapy also depends ona lesions perceived malignanttendencies as the potentialbenefits of radiation therapy andchemotherapy must be weighedagainst their morbidity

bull The decision to use postoperativeadjuvant therapy also depends ona lesions perceived malignanttendencies as the potentialbenefits of radiation therapy andchemotherapy must be weighedagainst their morbidity

10202016 24unusual giomas

Pleomorphic xanthoastrocytoma

bull First described by Kepsand colleuges in 1979

bull Represents lt1 of allastrocytomas

bull May be found alongentire neuraxis mostlysupratentorial( temporallobe)

bull Cerebellum and soinalcord are the other sites

bull Pts present in 2nd and 3rd

decadesbull Median age at 14 yearsbull Both sexes are affected

equallybull 70-80 pts present with

seizures and headachefocal neuro deficits

bull Rarely tumor mayhemmorrhage

10202016 25unusual giomas

bull Supratentorail are mostoften cortical and meningesbased

bull Chronic seizuresbull Grossely these are firm

tumors variable in colorrelatively avascular

bull Typically invade the piaarachnoid space

bull 13 pts have involvementof all the three meninges

bull Cyst fluid is xanthochromic

bull Very specific microscpoiccriteria for diagnosis are

1 Pleomorphic andxanthomatous astrocytes

2 Perivascular lymphocytes3 Non infilterating histology4 Reticulin stainingbull Pleomorphism can be as

vast as glioblastoma orsarcomatous tumors

10202016 26unusual giomas

bull IHC Staining revealsspindle cells and roundpleomorphic cellspositive for S-100 andGFAP

bull Staining forsynaptophysin and NSEmay be also present

bull Some workers havereported them to be asubtype of ganglioglioma

bull Association between theNeurofibromatosis Sturge Weber Syndrome

bull Loss of chr 9 and gain ofchromosome 7 have beenfound

10202016 27unusual giomas

Imaging

bull CTbull PXAs are typically hypo

or isodense and may bewell or poorlydemarcated usually withno or little surroundingoedema

bull Calcification is rarebull Due to its superficial

location it may causescalloping of theoverlying bone

10202016 28unusual giomas

bull MRIbull T1

ndash solid component iso tohypointense cf grey matter

ndash cystic component low signalndash leptomeningeal involvement

seen in over 70 of casesbull T1 C+ (Gd)

ndash solid component usuallyenhances vividly

bull T2ndash solid component iso to

hyperintense cf grey matterndash cystic component high signalndash on T2 FLAIR sequence cystic

areas show hyperintensityrelative to CSF due to higherprotein contents

ndash little surrounding vasogenicoedema

10202016 29unusual giomas

Management amp Outcome

bull Quite rare tumorsbull Surgical excision appears to

be best favoured modalitybull Actuarial survival trates are

91 at 5 years and 82 at10 years

bull Median survival 18 years

bull Total resection should beattempted in every case asfar as possible

bull Resection of cyst lining isnot indicated

bull Data regarding the use ofadjuvant therapy at thistime are limited to few casereports only

bull Pts who had incompleteresection high mitoticindex necrosis should bereserved for strict vigilanceand CEMRI on follow ups

10202016 30unusual giomas

bull At progression repeatresection be attempted

bull Proression should betaken as aggressivetumor type

bull Malignanttransformation maynecessitate adjuvanttherapy

10202016 31unusual giomas

bull Br J Neurosurg 2013 Dec27(6)759-64 doi 103109026886972013776666 Epub 2013 Mar 20

bull Pleomorphic xanthoastrocytoma long-term results of surgical treatment and analysis of prognosticfactors

bull Gallo P1 Cecchi PC Locatelli F Rizzo P Ghimenton C Gerosa M Pinna G

bull BACKGROUND

bull Pleomorphic Xanthoastrocytoma (PXA) is a rare brain tumour most commonly affecting children andyoung adults To date only few data regarding the long-term follow-up of these patients after surgery are

available The aim of this study is to describe our single-institution experience in the surgicalmanagement of this particular glioma over a period of over 18 years

bull METHODS

bull We performed a retrospective review of all cases of PXA (40 patients) operated upon at theDepartment of Neurosurgery of Verona Italy between 1990 and 2008 The impact of clinical radiologicalsurgical and histological factors on overall survival (OS) and progression-free survival (PFS) was analysed bymeans of univariate and multivariate models

bull FINDINGS

bull We achieved a gross total resection (GTR) in 65 of patients Histological diagnosis was of grade II in 80anaplastic features were present in the remaining 20 Adjuvant treatment radiotherapy or chemo-

radiotherapy was administered in 40 of the cases Median follow-up was 74 months OS at 5- and10 years was 7632 and 6824 respectively PFS at 5- and 10 years was 71 and 58respectively In the multivariate model histological grade extent of resection and age at diagnosis (le 30years vs gt 30 years) were the only independent prognostic factors for both OS and PFS

bull CONCLUSIONS

bull Our retrospective long-term study confirms the relatively favourable prognosis associatedwith PXA Young patients with a low-grade tumour (WHO grade II) who underwent GTR carrythe longest OS and PFS

10202016 32unusual giomas

bull Brain Tumor Pathol 2006 Apr23(1)55-63

bull A case of anaplastic pleomorphic xanthoastrocytoma presenting with tumor bleeding and cerebrospinal fluid dissemination

bull Asano K1 Miyamoto S Kubo O Kikkukawa T Yagihashi A Ohkuma H

bull Pleomorphic xanthoastrocytoma (PXA) has been considered an astrocytic tumorwith a relatively favorable prognosis However PXA cases having several recurrent patterns with poor prognosis have been reported in recent years and a new concept of anaplastic PXA has been proposed The present case was a 59-year-old woman who presented with tumor bleeding onset and cerebrospinal fluid dissemination The patient had sudden-onset right hemiparesis aphasia and consciousness disturbance and was admitted to a local area hospital After emergency surgery had removed the hematoma postoperative contrast-enhanced CT scan revealed a left temporal tumor A second surgery was therefore performed for initial tumor removal 2 months later Histopathological findings showed that the tumor was typical PXA with strong pleomorphism and xanthomatous changes and contained an ependymoma-like component in the center area However endothelial proliferation and mitosis were more remarkable compared to ordinary PXA The MIB-1 labeling index was 98 high From these findings the histopathological diagnosis was anaplastic PXA The patient underwent surgery to remove recurrent tumors 5 and 16 months later The patient died 36 months after the first onset and CT revealed glioblastoma-like

findings and cerebrospinal fluid dissemination This case report is the first case in which PXA presented with tumor bleeding onset Histopathological findings suggested anaplastic PXA from the first surgical specimens and PXA recurred many times We thus believe that the patient displayed primary anaplastic PXA rather than secondary anaplastic PXA that results in malignant transformation10202016 33unusual giomas

Ganglioglioma

bull WHO grade 1bull Initially described by

Perkins in 1926bull Benign well

differentiatedneuroepithelial tumors

bull Most commonly found intemporal lobe though canoccur anywhere in CNS

bull Commonly found inyoung patients withepilepsy

bull Most commonly notedtumor in temporal lobeepilepsy patients

bull Have been described inbrain steem spinal cordand cerebellum

bull Account for 1 ofprimary brain tumors

10202016 34unusual giomas

bull Male dominencebull Most common features

are seizures headachedizziness ataxiaprogressive weakness

bull Malignanttransformation is rare

bull Histology showsimmature mixture ofabnormal neural andglial elements

bull Macroscopically appearsolid or cystic

bull Cystic are welldelineatedfromsurrounding brain

bull Calcification is common

10202016 35unusual giomas

bull IHC staing shows reactivity for GFAP S-100 andvimentin

bull Neurons show synaptophysin class III betatubulin NFP chromogranin reactivity

10202016 36unusual giomas

Imaging

bull Highly variable imagingcharacters

bull No special typicalimaging criteria

bull Enhancement patternsare highly variable

bull Iso to hypointense onT1WI

bull Heterogenous andhyperintense on T2WI

bull Can contain solid cyticcalcified components

10202016 37unusual giomas

bull FDG PET scan showshypometabolism oreumetabolism in thesepatients

10202016 38unusual giomas

Management And Outcome

bull Gold standard is grosstotal resection

bull Seizure control aftersurgery is as high as80

bull Survival rates are highbull Event free survival of

low grade and highgrade gangiogioma are57 vs 15

bull The optimal surgicalresection depends uponthe eloquent areasinvolved in the vicinityof the tumor

bull The use ofchemotherapy is notwell established

bull Typically they shouldbe allowed for chemoand radiotherapy

10202016 39unusual giomas

bull The use of adjuvant therapy is warranted in thosecases which harbour residual lesions or features ofanaplasia

bull Potential harm to nearby brain areas preclude thejudicious use of radiation

10202016 40unusual giomas

bull Brain Tumor Res Treat 2014 Oct 2(2) 49ndash55

bull Treatment and Outcomes for Gangliogliomas A Single-Center Review of 16 Patients

bull Jye Young Song Jeong Hoon Kim Young Hyun Cho Chang Jin Kim and Eun Jung Lee

bull Ganglioglioma is a rare and slowly growing benign tumor We investigated the outcomes of patients who underwent different combination treatments

bull Methods

bull Between 1998 and 2012 16 patients including 11 men and 5 women with a median age of 125 years (range 25-65 years) were treated for intracranial gangliogliomas at our institution The median follow-up period was 57 years (range 48 days-156 years) Fifteen cases were included in the outcome assessment because one patient was lost to follow-up Complete resection was achieved in 8 (53) patients Six (40) patients underwent incomplete resection with or without adjuvant radiotherapy and one patient with a brainstem tumor underwent only stereotactic biopsy

bull Results

bull Gangliogliomas predominantly affected young (875) male patients and most frequently presented with seizures (64) Of eight patients who underwent complete resection seven did not show recurrence whereas only three of six with incomplete resection showed no recurrence Four patients with recurrence received salvage treatments (two repeat surgeries and two radiosurgeries) A tumor control rate of 93 (1415) was achieved at the last follow-up No recurrence or malignant changes were observed after a median follow-up of 12 and 45 years in four patients who received gamma knife (GK) radiosurgery as adjuvant and salvage treatment

bull Conclusion

bull Complete resection produced the best outcomes and incomplete resection followed by adjuvant or salvage treatments showed favorable outcomes In patients who are not eligible for complete resection because of tumor location or potential neurologic deficits following surgery GK radiosurgery should be considered for the treatment of residual or recurrent tumors

10202016 41unusual giomas

Papillary tumours of pineal

bull WHO grade II AND IIIbull Relatively new edition to WHObull They are equal in both sexesbull Reach medical attention due to headache mental

status changes and altered visionbull Contain eosinophilic cells with distinctive

borders with large pleomorphic nucleibull Positive for cytokeratin synaptophysin stain

variably for GFAP vimentin S-100

10202016 unusual giomas 42

Imaging

bull Generally found to be diffusely enhancinglesion of pineal region

bull Post 3rd ventricle with HCPbull Appear well demarcated from thalamus and

cerebellum

10202016 unusual giomas 43

Mangement

bull Standard treatment is excisionbull Grow slowly and donot transform to

malignant tumorsbull Local recurrence is however possiblebull Radiotherapy appears to provide good control

in local recurrence

10202016 unusual giomas 44

Choroid Glioma Of Third Ventricle

bull WHO Grade IIbull Ist recognised by WHO in

1998bull Arise in the anterior third

ventriclebull Commonly come to

attention after HCPbull Appera to contain in 3rd

ventricle and thought toarise from ventricularsurface

bull Composed of GFAP andvimentin positive cells inmucinous matrix

bull Nuclei are uniform insize and shape

bull Indolent growthbull May show EGF receptor

positivitybull Some suggets these tumor

arise from the lminaterminalis andventricular tanycytes

10202016 45unusual giomas

Imaging

bull Commonly fill 3rd

ventriclebull Diffusely enhance with

contrastbull Appear to infiltrate the

thalamusbull Large tumors may be

difficult to differentiatefrom suprasellar masses

10202016 46unusual giomas

10202016 47unusual giomas

bull Surgical management is limited by locationbull Access through the dialted ventricle and lamina

terminalis may be challangingbull Subtotal resection is thus commonbull Role of adjuvant radiotherapy is unknown

10202016 48unusual giomas

bull Acta Neurochir (Wien) 2008 Nov150(11)1183-91 doi 101007s00701-008-0014-6 Epub

2008 Oct 21

bull Chordoid glioma of the third ventricle

bull Vanhauwaert DJ1 Clement F Van Dorpe J Deruytter MJKGROUND

bull Chordoid glioma is a rare tumour (World Health Organisation grade II) originating from the third ventricle

with both glial and chordoid features It was first described by Brat in 1998 Since there is no detailed

information available on the outcome after surgery and adjuvant treatment we reviewed the literature

bull METHODS

bull A literature search through PUBMED revealed 50 cases of chordoid glioma Most reports

were found in pathology journals Information on the postoperative course was sometimes

very limited We reviewed the available literature and studied in detail the presenting

symptoms mortality and postoperative complications in relation to the extent of resective

surgery as well as the importance of adjuvant treatment

bull CONCLUSIONS

bull Mortality in the immediate postoperative period is 32 and is higher after gross total

resection as compared to subtotal resection Non-fatal postoperative complications are

hypothalamic disorders and mental alterations Gross total resection is the treatment of

choice since no recurrence has been reported after macroscopically complete resection

but this is often difficult because of the location and adherence to the hypothalamus The

role of postoperative radiotherapy is uncertain There is some indication that radiosurgery

with or without conventional irradiation is superior to conventional radiation alone Planned

subtotal resection followed by stereotactic radiosurgery can be a safe and effective

alternative in a patient in whom gross total resection is considered to be too risky

There is no report on the use of chemotherapy in the treatment of chordoid gliomas More

information about the optimal treatment strategy is needed and more reports are also needed10202016 49unusual giomas

bull Neurologist 2011 Jan17(1)52bull Chordoid glioma a case report and literature reviewbull Liu WP1 Cheng JX Yi XC Zhen HN Fei Z Li Q Zhang Xbull chordoid glioma is a rare tumor (World Health Organization grade II) with both

glial and chordoid features often located in the suprasellar region and anterior third ventricle It was first described by Brat in 1998 Because there is no detailed information available from the clinical perspective we reviewed the literature

bull METHODS

bull a literature search through PUBMED and CNKI revealed 64 cases of chordoid glioma Information on the clinical course was very limited We reviewed the literature and studied the pathologic and imaging features postoperative mortality and morbidity in relation to surgical extension and approaches and the importance of adjuvant treatment

bull CONCLUSIONS bull mortality in the immediate postoperative period is 28 and postoperative

morbidity is 60 which are statistically higher after gross total resection as compared with subtotal resection Translamina terminalis approach is considered to be the best approach The current study cannot document that patients have longer survival and higher quality of life after gross total resection than subtotal resection The role of postoperative radiotherapy is uncertain and there is no report on the use of chemotherapy More information about the optimal treatment strategy is needed

10202016 50unusual giomas

Conclusion

bull They are identified by their unique morphologicalfeatures

bull Many of these have indolent course and identified byepilepsy

bull Potentail diagnosis should be considered in youngpatients with seizures and cortically based lesions

bull Correct diagnosis requires experiencedneuropathologisrt

bull As more experienced is gained more knowledgetowards better understanding shall provide betteroutcomes in these patients

10202016 unusual giomas 51

10202016 52unusual giomas

Content supportndash youmann neurosurgery textbook

10202016 11unusual giomas

Management and outcome

bull Follow an indolent coursebull Rare progressionbull Surgical cure obtained by total resectionbull Aggressive forms are exceedingly rare

10202016 12unusual giomas

bull Perm J 2013 Winter17(1)e100-2 doi 107812TPP12-060

bull The treatment of angiocentric glioma case report and literature review

bull Alexandru D1 Haghighi B Muhonen MG

bull Abstract

bull Angiocentric glioma is a recently described tumor recognized since 2007

by the World Health Organization Classification of Tumours of the Central

Nervous System We present the only case of angiocentric glioma at our

institution in the last 15 years and review the literature in an attempt to

establish prognostic parameters Our search revealed only 27 cases of

angiocentric glioma in the literature The most common presenting

symptom of angiocentric glioma was seizures Gross total resection of

the lesion was curative without need for radiation or chemotherapy

10202016 13unusual giomas

Astroblastoma

bull WHO grade 1bull Initially described by Bailey

and Bucy in 1930bull Has been a matter of debate

for similar features asanaplastic astrocytomaglioblastoma andgemistocytic astrocytomas

bull Commonly found in 1st

three decades of lifebull Female

preponderance(111)

bull Affects the hemispheresinvolving cortex subcortical andperiventricular regions

bull No infratentorail tumorhave been reported

bull Pts present with features ofraised ICP cortical deficitsseizures personalitychanges

10202016 14unusual giomas

bull Greatly variable naturalhistory

bull Have a slow andindolent course

bull Rarely show anaggressive outcome

bull Almost always definedby their grossappearance

bull Cut surface revealhomogenous soft pinkgrey substances

bull Cystic areas arefrequently encountered

bull The clusters of cellsform pseudorossette

10202016 15unusual giomas

bull Richly supplied byblood vessels

bull Nuclei of tumor cellsare away from bloodvessels and send fineprocesse to the vesselwall

bull High number of mitoticfigures can be found

bull Cellular atypia isfrequently noted

bull On IHC tumor cellsstain positive for GFAP

bull Positivity for vimentinNSE S-1OO EMA

bull These are positive foranti leu 7 antibodiesspecific for cells ofepithelial origin

bull Central necrosis can befound

10202016 16unusual giomas

Imaging

bull On CT the appearnacesvaries from poorlydefined hypodensetumor to welldemarcated and highlyenhancing masses

10202016 17unusual giomas

bull On MR image tumorsare hypointense towhite matter on T1

bull Hyperintense on T2bull Hyperintense on FLAIRbull Enhancement is seen

around the cystic centre

10202016 18unusual giomas

Management And Prognosis

bull Surgical resection is main treatmentbull Gross total resection can be easily achieved as

lesions are mostly in the cortical areasbull Therapeutic value is of radiation and

chemotherapy is uncertain as these are raretumors

bull Prediction of outcome is difficult as these remainindolent for a long time and may rapidlytransform to glioblastoma resulting in fataloutcomes

10202016 19unusual giomas

Pilomyxoid astrocytoma

bull Recently described tumorsbull Similarities with pilocytic

astrocytomasbull Until recently these were

grouped with PAbull These involve entire

neuraxisbull Mean age at preentation is

18 monthsbull Most are found in

hypothalamic ndash chiasmicregion

bull Pts present with features ofraised ICP

bull RAISED HEADCIRCUMFERENCE may beonly subtle sign in infants

bull Histologically consists ofmyxoid matrix with cells inloose fibrillary and myxoidbackground

bull Tumor cells formperivascular rosettes

10202016 20unusual giomas

bull Preferred sites includebull (1) optic nerve (lsquooptic

nerve gliomarsquo)bull (2) optic

chiasmhypothalamusbull (3) thalamus and basal

gangliabull (4) cerebral hemispherebull (5) cerebellum (lsquocerebellar

astrocytomarsquo) andbull (6) brainstem (dorsal

exophytic brainstemglioma)

bull Genetically chracterized bythe presence of NF1mutations BRAFduplications and theabsence of IDH1 mutation

10202016 21unusual giomas

Imaging

bull The huge chiasmatic-hypothalamic tumor revealslow signal intensity on aT1-weighted image (A)

bull Bright signal intensity on aT2-weighted image (B)

bull Gadolinium-enhancedbrain MR images showheterogeneously strongenhancement (C and D)

bull Leptomeningealdisseminations at thecervical spine levels

10202016 22unusual giomas

bull NO standard careprotocols have beendefined

bull As these are newlydefined and raretumors

bull Surgical resection isfavoured if locationfavours

bull Mean progression freesurvival is 25 months

bull Mean overall survival is60 months

bull Reduced survival isbecause of inability toachieve gross resection

10202016 23unusual giomas

bull The usual indications foradjuvant treatment(chemotherapy orradiation therapy)include tumor recurrenceafter initial completeresection or symptomatictumors

bull In addition adjuvanttreatment is generallyindicated for tumors withgrowth on follow-upimaging even in theabsence of symptoms

bull The role of chemotherapyis in evolution

bull Chemotherapy may beimplemented for the treatment ofinoperable or partially resectedgliomas

bull The decision to use postoperativeadjuvant therapy also depends ona lesions perceived malignanttendencies as the potentialbenefits of radiation therapy andchemotherapy must be weighedagainst their morbidity

bull The decision to use postoperativeadjuvant therapy also depends ona lesions perceived malignanttendencies as the potentialbenefits of radiation therapy andchemotherapy must be weighedagainst their morbidity

10202016 24unusual giomas

Pleomorphic xanthoastrocytoma

bull First described by Kepsand colleuges in 1979

bull Represents lt1 of allastrocytomas

bull May be found alongentire neuraxis mostlysupratentorial( temporallobe)

bull Cerebellum and soinalcord are the other sites

bull Pts present in 2nd and 3rd

decadesbull Median age at 14 yearsbull Both sexes are affected

equallybull 70-80 pts present with

seizures and headachefocal neuro deficits

bull Rarely tumor mayhemmorrhage

10202016 25unusual giomas

bull Supratentorail are mostoften cortical and meningesbased

bull Chronic seizuresbull Grossely these are firm

tumors variable in colorrelatively avascular

bull Typically invade the piaarachnoid space

bull 13 pts have involvementof all the three meninges

bull Cyst fluid is xanthochromic

bull Very specific microscpoiccriteria for diagnosis are

1 Pleomorphic andxanthomatous astrocytes

2 Perivascular lymphocytes3 Non infilterating histology4 Reticulin stainingbull Pleomorphism can be as

vast as glioblastoma orsarcomatous tumors

10202016 26unusual giomas

bull IHC Staining revealsspindle cells and roundpleomorphic cellspositive for S-100 andGFAP

bull Staining forsynaptophysin and NSEmay be also present

bull Some workers havereported them to be asubtype of ganglioglioma

bull Association between theNeurofibromatosis Sturge Weber Syndrome

bull Loss of chr 9 and gain ofchromosome 7 have beenfound

10202016 27unusual giomas

Imaging

bull CTbull PXAs are typically hypo

or isodense and may bewell or poorlydemarcated usually withno or little surroundingoedema

bull Calcification is rarebull Due to its superficial

location it may causescalloping of theoverlying bone

10202016 28unusual giomas

bull MRIbull T1

ndash solid component iso tohypointense cf grey matter

ndash cystic component low signalndash leptomeningeal involvement

seen in over 70 of casesbull T1 C+ (Gd)

ndash solid component usuallyenhances vividly

bull T2ndash solid component iso to

hyperintense cf grey matterndash cystic component high signalndash on T2 FLAIR sequence cystic

areas show hyperintensityrelative to CSF due to higherprotein contents

ndash little surrounding vasogenicoedema

10202016 29unusual giomas

Management amp Outcome

bull Quite rare tumorsbull Surgical excision appears to

be best favoured modalitybull Actuarial survival trates are

91 at 5 years and 82 at10 years

bull Median survival 18 years

bull Total resection should beattempted in every case asfar as possible

bull Resection of cyst lining isnot indicated

bull Data regarding the use ofadjuvant therapy at thistime are limited to few casereports only

bull Pts who had incompleteresection high mitoticindex necrosis should bereserved for strict vigilanceand CEMRI on follow ups

10202016 30unusual giomas

bull At progression repeatresection be attempted

bull Proression should betaken as aggressivetumor type

bull Malignanttransformation maynecessitate adjuvanttherapy

10202016 31unusual giomas

bull Br J Neurosurg 2013 Dec27(6)759-64 doi 103109026886972013776666 Epub 2013 Mar 20

bull Pleomorphic xanthoastrocytoma long-term results of surgical treatment and analysis of prognosticfactors

bull Gallo P1 Cecchi PC Locatelli F Rizzo P Ghimenton C Gerosa M Pinna G

bull BACKGROUND

bull Pleomorphic Xanthoastrocytoma (PXA) is a rare brain tumour most commonly affecting children andyoung adults To date only few data regarding the long-term follow-up of these patients after surgery are

available The aim of this study is to describe our single-institution experience in the surgicalmanagement of this particular glioma over a period of over 18 years

bull METHODS

bull We performed a retrospective review of all cases of PXA (40 patients) operated upon at theDepartment of Neurosurgery of Verona Italy between 1990 and 2008 The impact of clinical radiologicalsurgical and histological factors on overall survival (OS) and progression-free survival (PFS) was analysed bymeans of univariate and multivariate models

bull FINDINGS

bull We achieved a gross total resection (GTR) in 65 of patients Histological diagnosis was of grade II in 80anaplastic features were present in the remaining 20 Adjuvant treatment radiotherapy or chemo-

radiotherapy was administered in 40 of the cases Median follow-up was 74 months OS at 5- and10 years was 7632 and 6824 respectively PFS at 5- and 10 years was 71 and 58respectively In the multivariate model histological grade extent of resection and age at diagnosis (le 30years vs gt 30 years) were the only independent prognostic factors for both OS and PFS

bull CONCLUSIONS

bull Our retrospective long-term study confirms the relatively favourable prognosis associatedwith PXA Young patients with a low-grade tumour (WHO grade II) who underwent GTR carrythe longest OS and PFS

10202016 32unusual giomas

bull Brain Tumor Pathol 2006 Apr23(1)55-63

bull A case of anaplastic pleomorphic xanthoastrocytoma presenting with tumor bleeding and cerebrospinal fluid dissemination

bull Asano K1 Miyamoto S Kubo O Kikkukawa T Yagihashi A Ohkuma H

bull Pleomorphic xanthoastrocytoma (PXA) has been considered an astrocytic tumorwith a relatively favorable prognosis However PXA cases having several recurrent patterns with poor prognosis have been reported in recent years and a new concept of anaplastic PXA has been proposed The present case was a 59-year-old woman who presented with tumor bleeding onset and cerebrospinal fluid dissemination The patient had sudden-onset right hemiparesis aphasia and consciousness disturbance and was admitted to a local area hospital After emergency surgery had removed the hematoma postoperative contrast-enhanced CT scan revealed a left temporal tumor A second surgery was therefore performed for initial tumor removal 2 months later Histopathological findings showed that the tumor was typical PXA with strong pleomorphism and xanthomatous changes and contained an ependymoma-like component in the center area However endothelial proliferation and mitosis were more remarkable compared to ordinary PXA The MIB-1 labeling index was 98 high From these findings the histopathological diagnosis was anaplastic PXA The patient underwent surgery to remove recurrent tumors 5 and 16 months later The patient died 36 months after the first onset and CT revealed glioblastoma-like

findings and cerebrospinal fluid dissemination This case report is the first case in which PXA presented with tumor bleeding onset Histopathological findings suggested anaplastic PXA from the first surgical specimens and PXA recurred many times We thus believe that the patient displayed primary anaplastic PXA rather than secondary anaplastic PXA that results in malignant transformation10202016 33unusual giomas

Ganglioglioma

bull WHO grade 1bull Initially described by

Perkins in 1926bull Benign well

differentiatedneuroepithelial tumors

bull Most commonly found intemporal lobe though canoccur anywhere in CNS

bull Commonly found inyoung patients withepilepsy

bull Most commonly notedtumor in temporal lobeepilepsy patients

bull Have been described inbrain steem spinal cordand cerebellum

bull Account for 1 ofprimary brain tumors

10202016 34unusual giomas

bull Male dominencebull Most common features

are seizures headachedizziness ataxiaprogressive weakness

bull Malignanttransformation is rare

bull Histology showsimmature mixture ofabnormal neural andglial elements

bull Macroscopically appearsolid or cystic

bull Cystic are welldelineatedfromsurrounding brain

bull Calcification is common

10202016 35unusual giomas

bull IHC staing shows reactivity for GFAP S-100 andvimentin

bull Neurons show synaptophysin class III betatubulin NFP chromogranin reactivity

10202016 36unusual giomas

Imaging

bull Highly variable imagingcharacters

bull No special typicalimaging criteria

bull Enhancement patternsare highly variable

bull Iso to hypointense onT1WI

bull Heterogenous andhyperintense on T2WI

bull Can contain solid cyticcalcified components

10202016 37unusual giomas

bull FDG PET scan showshypometabolism oreumetabolism in thesepatients

10202016 38unusual giomas

Management And Outcome

bull Gold standard is grosstotal resection

bull Seizure control aftersurgery is as high as80

bull Survival rates are highbull Event free survival of

low grade and highgrade gangiogioma are57 vs 15

bull The optimal surgicalresection depends uponthe eloquent areasinvolved in the vicinityof the tumor

bull The use ofchemotherapy is notwell established

bull Typically they shouldbe allowed for chemoand radiotherapy

10202016 39unusual giomas

bull The use of adjuvant therapy is warranted in thosecases which harbour residual lesions or features ofanaplasia

bull Potential harm to nearby brain areas preclude thejudicious use of radiation

10202016 40unusual giomas

bull Brain Tumor Res Treat 2014 Oct 2(2) 49ndash55

bull Treatment and Outcomes for Gangliogliomas A Single-Center Review of 16 Patients

bull Jye Young Song Jeong Hoon Kim Young Hyun Cho Chang Jin Kim and Eun Jung Lee

bull Ganglioglioma is a rare and slowly growing benign tumor We investigated the outcomes of patients who underwent different combination treatments

bull Methods

bull Between 1998 and 2012 16 patients including 11 men and 5 women with a median age of 125 years (range 25-65 years) were treated for intracranial gangliogliomas at our institution The median follow-up period was 57 years (range 48 days-156 years) Fifteen cases were included in the outcome assessment because one patient was lost to follow-up Complete resection was achieved in 8 (53) patients Six (40) patients underwent incomplete resection with or without adjuvant radiotherapy and one patient with a brainstem tumor underwent only stereotactic biopsy

bull Results

bull Gangliogliomas predominantly affected young (875) male patients and most frequently presented with seizures (64) Of eight patients who underwent complete resection seven did not show recurrence whereas only three of six with incomplete resection showed no recurrence Four patients with recurrence received salvage treatments (two repeat surgeries and two radiosurgeries) A tumor control rate of 93 (1415) was achieved at the last follow-up No recurrence or malignant changes were observed after a median follow-up of 12 and 45 years in four patients who received gamma knife (GK) radiosurgery as adjuvant and salvage treatment

bull Conclusion

bull Complete resection produced the best outcomes and incomplete resection followed by adjuvant or salvage treatments showed favorable outcomes In patients who are not eligible for complete resection because of tumor location or potential neurologic deficits following surgery GK radiosurgery should be considered for the treatment of residual or recurrent tumors

10202016 41unusual giomas

Papillary tumours of pineal

bull WHO grade II AND IIIbull Relatively new edition to WHObull They are equal in both sexesbull Reach medical attention due to headache mental

status changes and altered visionbull Contain eosinophilic cells with distinctive

borders with large pleomorphic nucleibull Positive for cytokeratin synaptophysin stain

variably for GFAP vimentin S-100

10202016 unusual giomas 42

Imaging

bull Generally found to be diffusely enhancinglesion of pineal region

bull Post 3rd ventricle with HCPbull Appear well demarcated from thalamus and

cerebellum

10202016 unusual giomas 43

Mangement

bull Standard treatment is excisionbull Grow slowly and donot transform to

malignant tumorsbull Local recurrence is however possiblebull Radiotherapy appears to provide good control

in local recurrence

10202016 unusual giomas 44

Choroid Glioma Of Third Ventricle

bull WHO Grade IIbull Ist recognised by WHO in

1998bull Arise in the anterior third

ventriclebull Commonly come to

attention after HCPbull Appera to contain in 3rd

ventricle and thought toarise from ventricularsurface

bull Composed of GFAP andvimentin positive cells inmucinous matrix

bull Nuclei are uniform insize and shape

bull Indolent growthbull May show EGF receptor

positivitybull Some suggets these tumor

arise from the lminaterminalis andventricular tanycytes

10202016 45unusual giomas

Imaging

bull Commonly fill 3rd

ventriclebull Diffusely enhance with

contrastbull Appear to infiltrate the

thalamusbull Large tumors may be

difficult to differentiatefrom suprasellar masses

10202016 46unusual giomas

10202016 47unusual giomas

bull Surgical management is limited by locationbull Access through the dialted ventricle and lamina

terminalis may be challangingbull Subtotal resection is thus commonbull Role of adjuvant radiotherapy is unknown

10202016 48unusual giomas

bull Acta Neurochir (Wien) 2008 Nov150(11)1183-91 doi 101007s00701-008-0014-6 Epub

2008 Oct 21

bull Chordoid glioma of the third ventricle

bull Vanhauwaert DJ1 Clement F Van Dorpe J Deruytter MJKGROUND

bull Chordoid glioma is a rare tumour (World Health Organisation grade II) originating from the third ventricle

with both glial and chordoid features It was first described by Brat in 1998 Since there is no detailed

information available on the outcome after surgery and adjuvant treatment we reviewed the literature

bull METHODS

bull A literature search through PUBMED revealed 50 cases of chordoid glioma Most reports

were found in pathology journals Information on the postoperative course was sometimes

very limited We reviewed the available literature and studied in detail the presenting

symptoms mortality and postoperative complications in relation to the extent of resective

surgery as well as the importance of adjuvant treatment

bull CONCLUSIONS

bull Mortality in the immediate postoperative period is 32 and is higher after gross total

resection as compared to subtotal resection Non-fatal postoperative complications are

hypothalamic disorders and mental alterations Gross total resection is the treatment of

choice since no recurrence has been reported after macroscopically complete resection

but this is often difficult because of the location and adherence to the hypothalamus The

role of postoperative radiotherapy is uncertain There is some indication that radiosurgery

with or without conventional irradiation is superior to conventional radiation alone Planned

subtotal resection followed by stereotactic radiosurgery can be a safe and effective

alternative in a patient in whom gross total resection is considered to be too risky

There is no report on the use of chemotherapy in the treatment of chordoid gliomas More

information about the optimal treatment strategy is needed and more reports are also needed10202016 49unusual giomas

bull Neurologist 2011 Jan17(1)52bull Chordoid glioma a case report and literature reviewbull Liu WP1 Cheng JX Yi XC Zhen HN Fei Z Li Q Zhang Xbull chordoid glioma is a rare tumor (World Health Organization grade II) with both

glial and chordoid features often located in the suprasellar region and anterior third ventricle It was first described by Brat in 1998 Because there is no detailed information available from the clinical perspective we reviewed the literature

bull METHODS

bull a literature search through PUBMED and CNKI revealed 64 cases of chordoid glioma Information on the clinical course was very limited We reviewed the literature and studied the pathologic and imaging features postoperative mortality and morbidity in relation to surgical extension and approaches and the importance of adjuvant treatment

bull CONCLUSIONS bull mortality in the immediate postoperative period is 28 and postoperative

morbidity is 60 which are statistically higher after gross total resection as compared with subtotal resection Translamina terminalis approach is considered to be the best approach The current study cannot document that patients have longer survival and higher quality of life after gross total resection than subtotal resection The role of postoperative radiotherapy is uncertain and there is no report on the use of chemotherapy More information about the optimal treatment strategy is needed

10202016 50unusual giomas

Conclusion

bull They are identified by their unique morphologicalfeatures

bull Many of these have indolent course and identified byepilepsy

bull Potentail diagnosis should be considered in youngpatients with seizures and cortically based lesions

bull Correct diagnosis requires experiencedneuropathologisrt

bull As more experienced is gained more knowledgetowards better understanding shall provide betteroutcomes in these patients

10202016 unusual giomas 51

10202016 52unusual giomas

Content supportndash youmann neurosurgery textbook

Management and outcome

bull Follow an indolent coursebull Rare progressionbull Surgical cure obtained by total resectionbull Aggressive forms are exceedingly rare

10202016 12unusual giomas

bull Perm J 2013 Winter17(1)e100-2 doi 107812TPP12-060

bull The treatment of angiocentric glioma case report and literature review

bull Alexandru D1 Haghighi B Muhonen MG

bull Abstract

bull Angiocentric glioma is a recently described tumor recognized since 2007

by the World Health Organization Classification of Tumours of the Central

Nervous System We present the only case of angiocentric glioma at our

institution in the last 15 years and review the literature in an attempt to

establish prognostic parameters Our search revealed only 27 cases of

angiocentric glioma in the literature The most common presenting

symptom of angiocentric glioma was seizures Gross total resection of

the lesion was curative without need for radiation or chemotherapy

10202016 13unusual giomas

Astroblastoma

bull WHO grade 1bull Initially described by Bailey

and Bucy in 1930bull Has been a matter of debate

for similar features asanaplastic astrocytomaglioblastoma andgemistocytic astrocytomas

bull Commonly found in 1st

three decades of lifebull Female

preponderance(111)

bull Affects the hemispheresinvolving cortex subcortical andperiventricular regions

bull No infratentorail tumorhave been reported

bull Pts present with features ofraised ICP cortical deficitsseizures personalitychanges

10202016 14unusual giomas

bull Greatly variable naturalhistory

bull Have a slow andindolent course

bull Rarely show anaggressive outcome

bull Almost always definedby their grossappearance

bull Cut surface revealhomogenous soft pinkgrey substances

bull Cystic areas arefrequently encountered

bull The clusters of cellsform pseudorossette

10202016 15unusual giomas

bull Richly supplied byblood vessels

bull Nuclei of tumor cellsare away from bloodvessels and send fineprocesse to the vesselwall

bull High number of mitoticfigures can be found

bull Cellular atypia isfrequently noted

bull On IHC tumor cellsstain positive for GFAP

bull Positivity for vimentinNSE S-1OO EMA

bull These are positive foranti leu 7 antibodiesspecific for cells ofepithelial origin

bull Central necrosis can befound

10202016 16unusual giomas

Imaging

bull On CT the appearnacesvaries from poorlydefined hypodensetumor to welldemarcated and highlyenhancing masses

10202016 17unusual giomas

bull On MR image tumorsare hypointense towhite matter on T1

bull Hyperintense on T2bull Hyperintense on FLAIRbull Enhancement is seen

around the cystic centre

10202016 18unusual giomas

Management And Prognosis

bull Surgical resection is main treatmentbull Gross total resection can be easily achieved as

lesions are mostly in the cortical areasbull Therapeutic value is of radiation and

chemotherapy is uncertain as these are raretumors

bull Prediction of outcome is difficult as these remainindolent for a long time and may rapidlytransform to glioblastoma resulting in fataloutcomes

10202016 19unusual giomas

Pilomyxoid astrocytoma

bull Recently described tumorsbull Similarities with pilocytic

astrocytomasbull Until recently these were

grouped with PAbull These involve entire

neuraxisbull Mean age at preentation is

18 monthsbull Most are found in

hypothalamic ndash chiasmicregion

bull Pts present with features ofraised ICP

bull RAISED HEADCIRCUMFERENCE may beonly subtle sign in infants

bull Histologically consists ofmyxoid matrix with cells inloose fibrillary and myxoidbackground

bull Tumor cells formperivascular rosettes

10202016 20unusual giomas

bull Preferred sites includebull (1) optic nerve (lsquooptic

nerve gliomarsquo)bull (2) optic

chiasmhypothalamusbull (3) thalamus and basal

gangliabull (4) cerebral hemispherebull (5) cerebellum (lsquocerebellar

astrocytomarsquo) andbull (6) brainstem (dorsal

exophytic brainstemglioma)

bull Genetically chracterized bythe presence of NF1mutations BRAFduplications and theabsence of IDH1 mutation

10202016 21unusual giomas

Imaging

bull The huge chiasmatic-hypothalamic tumor revealslow signal intensity on aT1-weighted image (A)

bull Bright signal intensity on aT2-weighted image (B)

bull Gadolinium-enhancedbrain MR images showheterogeneously strongenhancement (C and D)

bull Leptomeningealdisseminations at thecervical spine levels

10202016 22unusual giomas

bull NO standard careprotocols have beendefined

bull As these are newlydefined and raretumors

bull Surgical resection isfavoured if locationfavours

bull Mean progression freesurvival is 25 months

bull Mean overall survival is60 months

bull Reduced survival isbecause of inability toachieve gross resection

10202016 23unusual giomas

bull The usual indications foradjuvant treatment(chemotherapy orradiation therapy)include tumor recurrenceafter initial completeresection or symptomatictumors

bull In addition adjuvanttreatment is generallyindicated for tumors withgrowth on follow-upimaging even in theabsence of symptoms

bull The role of chemotherapyis in evolution

bull Chemotherapy may beimplemented for the treatment ofinoperable or partially resectedgliomas

bull The decision to use postoperativeadjuvant therapy also depends ona lesions perceived malignanttendencies as the potentialbenefits of radiation therapy andchemotherapy must be weighedagainst their morbidity

bull The decision to use postoperativeadjuvant therapy also depends ona lesions perceived malignanttendencies as the potentialbenefits of radiation therapy andchemotherapy must be weighedagainst their morbidity

10202016 24unusual giomas

Pleomorphic xanthoastrocytoma

bull First described by Kepsand colleuges in 1979

bull Represents lt1 of allastrocytomas

bull May be found alongentire neuraxis mostlysupratentorial( temporallobe)

bull Cerebellum and soinalcord are the other sites

bull Pts present in 2nd and 3rd

decadesbull Median age at 14 yearsbull Both sexes are affected

equallybull 70-80 pts present with

seizures and headachefocal neuro deficits

bull Rarely tumor mayhemmorrhage

10202016 25unusual giomas

bull Supratentorail are mostoften cortical and meningesbased

bull Chronic seizuresbull Grossely these are firm

tumors variable in colorrelatively avascular

bull Typically invade the piaarachnoid space

bull 13 pts have involvementof all the three meninges

bull Cyst fluid is xanthochromic

bull Very specific microscpoiccriteria for diagnosis are

1 Pleomorphic andxanthomatous astrocytes

2 Perivascular lymphocytes3 Non infilterating histology4 Reticulin stainingbull Pleomorphism can be as

vast as glioblastoma orsarcomatous tumors

10202016 26unusual giomas

bull IHC Staining revealsspindle cells and roundpleomorphic cellspositive for S-100 andGFAP

bull Staining forsynaptophysin and NSEmay be also present

bull Some workers havereported them to be asubtype of ganglioglioma

bull Association between theNeurofibromatosis Sturge Weber Syndrome

bull Loss of chr 9 and gain ofchromosome 7 have beenfound

10202016 27unusual giomas

Imaging

bull CTbull PXAs are typically hypo

or isodense and may bewell or poorlydemarcated usually withno or little surroundingoedema

bull Calcification is rarebull Due to its superficial

location it may causescalloping of theoverlying bone

10202016 28unusual giomas

bull MRIbull T1

ndash solid component iso tohypointense cf grey matter

ndash cystic component low signalndash leptomeningeal involvement

seen in over 70 of casesbull T1 C+ (Gd)

ndash solid component usuallyenhances vividly

bull T2ndash solid component iso to

hyperintense cf grey matterndash cystic component high signalndash on T2 FLAIR sequence cystic

areas show hyperintensityrelative to CSF due to higherprotein contents

ndash little surrounding vasogenicoedema

10202016 29unusual giomas

Management amp Outcome

bull Quite rare tumorsbull Surgical excision appears to

be best favoured modalitybull Actuarial survival trates are

91 at 5 years and 82 at10 years

bull Median survival 18 years

bull Total resection should beattempted in every case asfar as possible

bull Resection of cyst lining isnot indicated

bull Data regarding the use ofadjuvant therapy at thistime are limited to few casereports only

bull Pts who had incompleteresection high mitoticindex necrosis should bereserved for strict vigilanceand CEMRI on follow ups

10202016 30unusual giomas

bull At progression repeatresection be attempted

bull Proression should betaken as aggressivetumor type

bull Malignanttransformation maynecessitate adjuvanttherapy

10202016 31unusual giomas

bull Br J Neurosurg 2013 Dec27(6)759-64 doi 103109026886972013776666 Epub 2013 Mar 20

bull Pleomorphic xanthoastrocytoma long-term results of surgical treatment and analysis of prognosticfactors

bull Gallo P1 Cecchi PC Locatelli F Rizzo P Ghimenton C Gerosa M Pinna G

bull BACKGROUND

bull Pleomorphic Xanthoastrocytoma (PXA) is a rare brain tumour most commonly affecting children andyoung adults To date only few data regarding the long-term follow-up of these patients after surgery are

available The aim of this study is to describe our single-institution experience in the surgicalmanagement of this particular glioma over a period of over 18 years

bull METHODS

bull We performed a retrospective review of all cases of PXA (40 patients) operated upon at theDepartment of Neurosurgery of Verona Italy between 1990 and 2008 The impact of clinical radiologicalsurgical and histological factors on overall survival (OS) and progression-free survival (PFS) was analysed bymeans of univariate and multivariate models

bull FINDINGS

bull We achieved a gross total resection (GTR) in 65 of patients Histological diagnosis was of grade II in 80anaplastic features were present in the remaining 20 Adjuvant treatment radiotherapy or chemo-

radiotherapy was administered in 40 of the cases Median follow-up was 74 months OS at 5- and10 years was 7632 and 6824 respectively PFS at 5- and 10 years was 71 and 58respectively In the multivariate model histological grade extent of resection and age at diagnosis (le 30years vs gt 30 years) were the only independent prognostic factors for both OS and PFS

bull CONCLUSIONS

bull Our retrospective long-term study confirms the relatively favourable prognosis associatedwith PXA Young patients with a low-grade tumour (WHO grade II) who underwent GTR carrythe longest OS and PFS

10202016 32unusual giomas

bull Brain Tumor Pathol 2006 Apr23(1)55-63

bull A case of anaplastic pleomorphic xanthoastrocytoma presenting with tumor bleeding and cerebrospinal fluid dissemination

bull Asano K1 Miyamoto S Kubo O Kikkukawa T Yagihashi A Ohkuma H

bull Pleomorphic xanthoastrocytoma (PXA) has been considered an astrocytic tumorwith a relatively favorable prognosis However PXA cases having several recurrent patterns with poor prognosis have been reported in recent years and a new concept of anaplastic PXA has been proposed The present case was a 59-year-old woman who presented with tumor bleeding onset and cerebrospinal fluid dissemination The patient had sudden-onset right hemiparesis aphasia and consciousness disturbance and was admitted to a local area hospital After emergency surgery had removed the hematoma postoperative contrast-enhanced CT scan revealed a left temporal tumor A second surgery was therefore performed for initial tumor removal 2 months later Histopathological findings showed that the tumor was typical PXA with strong pleomorphism and xanthomatous changes and contained an ependymoma-like component in the center area However endothelial proliferation and mitosis were more remarkable compared to ordinary PXA The MIB-1 labeling index was 98 high From these findings the histopathological diagnosis was anaplastic PXA The patient underwent surgery to remove recurrent tumors 5 and 16 months later The patient died 36 months after the first onset and CT revealed glioblastoma-like

findings and cerebrospinal fluid dissemination This case report is the first case in which PXA presented with tumor bleeding onset Histopathological findings suggested anaplastic PXA from the first surgical specimens and PXA recurred many times We thus believe that the patient displayed primary anaplastic PXA rather than secondary anaplastic PXA that results in malignant transformation10202016 33unusual giomas

Ganglioglioma

bull WHO grade 1bull Initially described by

Perkins in 1926bull Benign well

differentiatedneuroepithelial tumors

bull Most commonly found intemporal lobe though canoccur anywhere in CNS

bull Commonly found inyoung patients withepilepsy

bull Most commonly notedtumor in temporal lobeepilepsy patients

bull Have been described inbrain steem spinal cordand cerebellum

bull Account for 1 ofprimary brain tumors

10202016 34unusual giomas

bull Male dominencebull Most common features

are seizures headachedizziness ataxiaprogressive weakness

bull Malignanttransformation is rare

bull Histology showsimmature mixture ofabnormal neural andglial elements

bull Macroscopically appearsolid or cystic

bull Cystic are welldelineatedfromsurrounding brain

bull Calcification is common

10202016 35unusual giomas

bull IHC staing shows reactivity for GFAP S-100 andvimentin

bull Neurons show synaptophysin class III betatubulin NFP chromogranin reactivity

10202016 36unusual giomas

Imaging

bull Highly variable imagingcharacters

bull No special typicalimaging criteria

bull Enhancement patternsare highly variable

bull Iso to hypointense onT1WI

bull Heterogenous andhyperintense on T2WI

bull Can contain solid cyticcalcified components

10202016 37unusual giomas

bull FDG PET scan showshypometabolism oreumetabolism in thesepatients

10202016 38unusual giomas

Management And Outcome

bull Gold standard is grosstotal resection

bull Seizure control aftersurgery is as high as80

bull Survival rates are highbull Event free survival of

low grade and highgrade gangiogioma are57 vs 15

bull The optimal surgicalresection depends uponthe eloquent areasinvolved in the vicinityof the tumor

bull The use ofchemotherapy is notwell established

bull Typically they shouldbe allowed for chemoand radiotherapy

10202016 39unusual giomas

bull The use of adjuvant therapy is warranted in thosecases which harbour residual lesions or features ofanaplasia

bull Potential harm to nearby brain areas preclude thejudicious use of radiation

10202016 40unusual giomas

bull Brain Tumor Res Treat 2014 Oct 2(2) 49ndash55

bull Treatment and Outcomes for Gangliogliomas A Single-Center Review of 16 Patients

bull Jye Young Song Jeong Hoon Kim Young Hyun Cho Chang Jin Kim and Eun Jung Lee

bull Ganglioglioma is a rare and slowly growing benign tumor We investigated the outcomes of patients who underwent different combination treatments

bull Methods

bull Between 1998 and 2012 16 patients including 11 men and 5 women with a median age of 125 years (range 25-65 years) were treated for intracranial gangliogliomas at our institution The median follow-up period was 57 years (range 48 days-156 years) Fifteen cases were included in the outcome assessment because one patient was lost to follow-up Complete resection was achieved in 8 (53) patients Six (40) patients underwent incomplete resection with or without adjuvant radiotherapy and one patient with a brainstem tumor underwent only stereotactic biopsy

bull Results

bull Gangliogliomas predominantly affected young (875) male patients and most frequently presented with seizures (64) Of eight patients who underwent complete resection seven did not show recurrence whereas only three of six with incomplete resection showed no recurrence Four patients with recurrence received salvage treatments (two repeat surgeries and two radiosurgeries) A tumor control rate of 93 (1415) was achieved at the last follow-up No recurrence or malignant changes were observed after a median follow-up of 12 and 45 years in four patients who received gamma knife (GK) radiosurgery as adjuvant and salvage treatment

bull Conclusion

bull Complete resection produced the best outcomes and incomplete resection followed by adjuvant or salvage treatments showed favorable outcomes In patients who are not eligible for complete resection because of tumor location or potential neurologic deficits following surgery GK radiosurgery should be considered for the treatment of residual or recurrent tumors

10202016 41unusual giomas

Papillary tumours of pineal

bull WHO grade II AND IIIbull Relatively new edition to WHObull They are equal in both sexesbull Reach medical attention due to headache mental

status changes and altered visionbull Contain eosinophilic cells with distinctive

borders with large pleomorphic nucleibull Positive for cytokeratin synaptophysin stain

variably for GFAP vimentin S-100

10202016 unusual giomas 42

Imaging

bull Generally found to be diffusely enhancinglesion of pineal region

bull Post 3rd ventricle with HCPbull Appear well demarcated from thalamus and

cerebellum

10202016 unusual giomas 43

Mangement

bull Standard treatment is excisionbull Grow slowly and donot transform to

malignant tumorsbull Local recurrence is however possiblebull Radiotherapy appears to provide good control

in local recurrence

10202016 unusual giomas 44

Choroid Glioma Of Third Ventricle

bull WHO Grade IIbull Ist recognised by WHO in

1998bull Arise in the anterior third

ventriclebull Commonly come to

attention after HCPbull Appera to contain in 3rd

ventricle and thought toarise from ventricularsurface

bull Composed of GFAP andvimentin positive cells inmucinous matrix

bull Nuclei are uniform insize and shape

bull Indolent growthbull May show EGF receptor

positivitybull Some suggets these tumor

arise from the lminaterminalis andventricular tanycytes

10202016 45unusual giomas

Imaging

bull Commonly fill 3rd

ventriclebull Diffusely enhance with

contrastbull Appear to infiltrate the

thalamusbull Large tumors may be

difficult to differentiatefrom suprasellar masses

10202016 46unusual giomas

10202016 47unusual giomas

bull Surgical management is limited by locationbull Access through the dialted ventricle and lamina

terminalis may be challangingbull Subtotal resection is thus commonbull Role of adjuvant radiotherapy is unknown

10202016 48unusual giomas

bull Acta Neurochir (Wien) 2008 Nov150(11)1183-91 doi 101007s00701-008-0014-6 Epub

2008 Oct 21

bull Chordoid glioma of the third ventricle

bull Vanhauwaert DJ1 Clement F Van Dorpe J Deruytter MJKGROUND

bull Chordoid glioma is a rare tumour (World Health Organisation grade II) originating from the third ventricle

with both glial and chordoid features It was first described by Brat in 1998 Since there is no detailed

information available on the outcome after surgery and adjuvant treatment we reviewed the literature

bull METHODS

bull A literature search through PUBMED revealed 50 cases of chordoid glioma Most reports

were found in pathology journals Information on the postoperative course was sometimes

very limited We reviewed the available literature and studied in detail the presenting

symptoms mortality and postoperative complications in relation to the extent of resective

surgery as well as the importance of adjuvant treatment

bull CONCLUSIONS

bull Mortality in the immediate postoperative period is 32 and is higher after gross total

resection as compared to subtotal resection Non-fatal postoperative complications are

hypothalamic disorders and mental alterations Gross total resection is the treatment of

choice since no recurrence has been reported after macroscopically complete resection

but this is often difficult because of the location and adherence to the hypothalamus The

role of postoperative radiotherapy is uncertain There is some indication that radiosurgery

with or without conventional irradiation is superior to conventional radiation alone Planned

subtotal resection followed by stereotactic radiosurgery can be a safe and effective

alternative in a patient in whom gross total resection is considered to be too risky

There is no report on the use of chemotherapy in the treatment of chordoid gliomas More

information about the optimal treatment strategy is needed and more reports are also needed10202016 49unusual giomas

bull Neurologist 2011 Jan17(1)52bull Chordoid glioma a case report and literature reviewbull Liu WP1 Cheng JX Yi XC Zhen HN Fei Z Li Q Zhang Xbull chordoid glioma is a rare tumor (World Health Organization grade II) with both

glial and chordoid features often located in the suprasellar region and anterior third ventricle It was first described by Brat in 1998 Because there is no detailed information available from the clinical perspective we reviewed the literature

bull METHODS

bull a literature search through PUBMED and CNKI revealed 64 cases of chordoid glioma Information on the clinical course was very limited We reviewed the literature and studied the pathologic and imaging features postoperative mortality and morbidity in relation to surgical extension and approaches and the importance of adjuvant treatment

bull CONCLUSIONS bull mortality in the immediate postoperative period is 28 and postoperative

morbidity is 60 which are statistically higher after gross total resection as compared with subtotal resection Translamina terminalis approach is considered to be the best approach The current study cannot document that patients have longer survival and higher quality of life after gross total resection than subtotal resection The role of postoperative radiotherapy is uncertain and there is no report on the use of chemotherapy More information about the optimal treatment strategy is needed

10202016 50unusual giomas

Conclusion

bull They are identified by their unique morphologicalfeatures

bull Many of these have indolent course and identified byepilepsy

bull Potentail diagnosis should be considered in youngpatients with seizures and cortically based lesions

bull Correct diagnosis requires experiencedneuropathologisrt

bull As more experienced is gained more knowledgetowards better understanding shall provide betteroutcomes in these patients

10202016 unusual giomas 51

10202016 52unusual giomas

Content supportndash youmann neurosurgery textbook

bull Perm J 2013 Winter17(1)e100-2 doi 107812TPP12-060

bull The treatment of angiocentric glioma case report and literature review

bull Alexandru D1 Haghighi B Muhonen MG

bull Abstract

bull Angiocentric glioma is a recently described tumor recognized since 2007

by the World Health Organization Classification of Tumours of the Central

Nervous System We present the only case of angiocentric glioma at our

institution in the last 15 years and review the literature in an attempt to

establish prognostic parameters Our search revealed only 27 cases of

angiocentric glioma in the literature The most common presenting

symptom of angiocentric glioma was seizures Gross total resection of

the lesion was curative without need for radiation or chemotherapy

10202016 13unusual giomas

Astroblastoma

bull WHO grade 1bull Initially described by Bailey

and Bucy in 1930bull Has been a matter of debate

for similar features asanaplastic astrocytomaglioblastoma andgemistocytic astrocytomas

bull Commonly found in 1st

three decades of lifebull Female

preponderance(111)

bull Affects the hemispheresinvolving cortex subcortical andperiventricular regions

bull No infratentorail tumorhave been reported

bull Pts present with features ofraised ICP cortical deficitsseizures personalitychanges

10202016 14unusual giomas

bull Greatly variable naturalhistory

bull Have a slow andindolent course

bull Rarely show anaggressive outcome

bull Almost always definedby their grossappearance

bull Cut surface revealhomogenous soft pinkgrey substances

bull Cystic areas arefrequently encountered

bull The clusters of cellsform pseudorossette

10202016 15unusual giomas

bull Richly supplied byblood vessels

bull Nuclei of tumor cellsare away from bloodvessels and send fineprocesse to the vesselwall

bull High number of mitoticfigures can be found

bull Cellular atypia isfrequently noted

bull On IHC tumor cellsstain positive for GFAP

bull Positivity for vimentinNSE S-1OO EMA

bull These are positive foranti leu 7 antibodiesspecific for cells ofepithelial origin

bull Central necrosis can befound

10202016 16unusual giomas

Imaging

bull On CT the appearnacesvaries from poorlydefined hypodensetumor to welldemarcated and highlyenhancing masses

10202016 17unusual giomas

bull On MR image tumorsare hypointense towhite matter on T1

bull Hyperintense on T2bull Hyperintense on FLAIRbull Enhancement is seen

around the cystic centre

10202016 18unusual giomas

Management And Prognosis

bull Surgical resection is main treatmentbull Gross total resection can be easily achieved as

lesions are mostly in the cortical areasbull Therapeutic value is of radiation and

chemotherapy is uncertain as these are raretumors

bull Prediction of outcome is difficult as these remainindolent for a long time and may rapidlytransform to glioblastoma resulting in fataloutcomes

10202016 19unusual giomas

Pilomyxoid astrocytoma

bull Recently described tumorsbull Similarities with pilocytic

astrocytomasbull Until recently these were

grouped with PAbull These involve entire

neuraxisbull Mean age at preentation is

18 monthsbull Most are found in

hypothalamic ndash chiasmicregion

bull Pts present with features ofraised ICP

bull RAISED HEADCIRCUMFERENCE may beonly subtle sign in infants

bull Histologically consists ofmyxoid matrix with cells inloose fibrillary and myxoidbackground

bull Tumor cells formperivascular rosettes

10202016 20unusual giomas

bull Preferred sites includebull (1) optic nerve (lsquooptic

nerve gliomarsquo)bull (2) optic

chiasmhypothalamusbull (3) thalamus and basal

gangliabull (4) cerebral hemispherebull (5) cerebellum (lsquocerebellar

astrocytomarsquo) andbull (6) brainstem (dorsal

exophytic brainstemglioma)

bull Genetically chracterized bythe presence of NF1mutations BRAFduplications and theabsence of IDH1 mutation

10202016 21unusual giomas

Imaging

bull The huge chiasmatic-hypothalamic tumor revealslow signal intensity on aT1-weighted image (A)

bull Bright signal intensity on aT2-weighted image (B)

bull Gadolinium-enhancedbrain MR images showheterogeneously strongenhancement (C and D)

bull Leptomeningealdisseminations at thecervical spine levels

10202016 22unusual giomas

bull NO standard careprotocols have beendefined

bull As these are newlydefined and raretumors

bull Surgical resection isfavoured if locationfavours

bull Mean progression freesurvival is 25 months

bull Mean overall survival is60 months

bull Reduced survival isbecause of inability toachieve gross resection

10202016 23unusual giomas

bull The usual indications foradjuvant treatment(chemotherapy orradiation therapy)include tumor recurrenceafter initial completeresection or symptomatictumors

bull In addition adjuvanttreatment is generallyindicated for tumors withgrowth on follow-upimaging even in theabsence of symptoms

bull The role of chemotherapyis in evolution

bull Chemotherapy may beimplemented for the treatment ofinoperable or partially resectedgliomas

bull The decision to use postoperativeadjuvant therapy also depends ona lesions perceived malignanttendencies as the potentialbenefits of radiation therapy andchemotherapy must be weighedagainst their morbidity

bull The decision to use postoperativeadjuvant therapy also depends ona lesions perceived malignanttendencies as the potentialbenefits of radiation therapy andchemotherapy must be weighedagainst their morbidity

10202016 24unusual giomas

Pleomorphic xanthoastrocytoma

bull First described by Kepsand colleuges in 1979

bull Represents lt1 of allastrocytomas

bull May be found alongentire neuraxis mostlysupratentorial( temporallobe)

bull Cerebellum and soinalcord are the other sites

bull Pts present in 2nd and 3rd

decadesbull Median age at 14 yearsbull Both sexes are affected

equallybull 70-80 pts present with

seizures and headachefocal neuro deficits

bull Rarely tumor mayhemmorrhage

10202016 25unusual giomas

bull Supratentorail are mostoften cortical and meningesbased

bull Chronic seizuresbull Grossely these are firm

tumors variable in colorrelatively avascular

bull Typically invade the piaarachnoid space

bull 13 pts have involvementof all the three meninges

bull Cyst fluid is xanthochromic

bull Very specific microscpoiccriteria for diagnosis are

1 Pleomorphic andxanthomatous astrocytes

2 Perivascular lymphocytes3 Non infilterating histology4 Reticulin stainingbull Pleomorphism can be as

vast as glioblastoma orsarcomatous tumors

10202016 26unusual giomas

bull IHC Staining revealsspindle cells and roundpleomorphic cellspositive for S-100 andGFAP

bull Staining forsynaptophysin and NSEmay be also present

bull Some workers havereported them to be asubtype of ganglioglioma

bull Association between theNeurofibromatosis Sturge Weber Syndrome

bull Loss of chr 9 and gain ofchromosome 7 have beenfound

10202016 27unusual giomas

Imaging

bull CTbull PXAs are typically hypo

or isodense and may bewell or poorlydemarcated usually withno or little surroundingoedema

bull Calcification is rarebull Due to its superficial

location it may causescalloping of theoverlying bone

10202016 28unusual giomas

bull MRIbull T1

ndash solid component iso tohypointense cf grey matter

ndash cystic component low signalndash leptomeningeal involvement

seen in over 70 of casesbull T1 C+ (Gd)

ndash solid component usuallyenhances vividly

bull T2ndash solid component iso to

hyperintense cf grey matterndash cystic component high signalndash on T2 FLAIR sequence cystic

areas show hyperintensityrelative to CSF due to higherprotein contents

ndash little surrounding vasogenicoedema

10202016 29unusual giomas

Management amp Outcome

bull Quite rare tumorsbull Surgical excision appears to

be best favoured modalitybull Actuarial survival trates are

91 at 5 years and 82 at10 years

bull Median survival 18 years

bull Total resection should beattempted in every case asfar as possible

bull Resection of cyst lining isnot indicated

bull Data regarding the use ofadjuvant therapy at thistime are limited to few casereports only

bull Pts who had incompleteresection high mitoticindex necrosis should bereserved for strict vigilanceand CEMRI on follow ups

10202016 30unusual giomas

bull At progression repeatresection be attempted

bull Proression should betaken as aggressivetumor type

bull Malignanttransformation maynecessitate adjuvanttherapy

10202016 31unusual giomas

bull Br J Neurosurg 2013 Dec27(6)759-64 doi 103109026886972013776666 Epub 2013 Mar 20

bull Pleomorphic xanthoastrocytoma long-term results of surgical treatment and analysis of prognosticfactors

bull Gallo P1 Cecchi PC Locatelli F Rizzo P Ghimenton C Gerosa M Pinna G

bull BACKGROUND

bull Pleomorphic Xanthoastrocytoma (PXA) is a rare brain tumour most commonly affecting children andyoung adults To date only few data regarding the long-term follow-up of these patients after surgery are

available The aim of this study is to describe our single-institution experience in the surgicalmanagement of this particular glioma over a period of over 18 years

bull METHODS

bull We performed a retrospective review of all cases of PXA (40 patients) operated upon at theDepartment of Neurosurgery of Verona Italy between 1990 and 2008 The impact of clinical radiologicalsurgical and histological factors on overall survival (OS) and progression-free survival (PFS) was analysed bymeans of univariate and multivariate models

bull FINDINGS

bull We achieved a gross total resection (GTR) in 65 of patients Histological diagnosis was of grade II in 80anaplastic features were present in the remaining 20 Adjuvant treatment radiotherapy or chemo-

radiotherapy was administered in 40 of the cases Median follow-up was 74 months OS at 5- and10 years was 7632 and 6824 respectively PFS at 5- and 10 years was 71 and 58respectively In the multivariate model histological grade extent of resection and age at diagnosis (le 30years vs gt 30 years) were the only independent prognostic factors for both OS and PFS

bull CONCLUSIONS

bull Our retrospective long-term study confirms the relatively favourable prognosis associatedwith PXA Young patients with a low-grade tumour (WHO grade II) who underwent GTR carrythe longest OS and PFS

10202016 32unusual giomas

bull Brain Tumor Pathol 2006 Apr23(1)55-63

bull A case of anaplastic pleomorphic xanthoastrocytoma presenting with tumor bleeding and cerebrospinal fluid dissemination

bull Asano K1 Miyamoto S Kubo O Kikkukawa T Yagihashi A Ohkuma H

bull Pleomorphic xanthoastrocytoma (PXA) has been considered an astrocytic tumorwith a relatively favorable prognosis However PXA cases having several recurrent patterns with poor prognosis have been reported in recent years and a new concept of anaplastic PXA has been proposed The present case was a 59-year-old woman who presented with tumor bleeding onset and cerebrospinal fluid dissemination The patient had sudden-onset right hemiparesis aphasia and consciousness disturbance and was admitted to a local area hospital After emergency surgery had removed the hematoma postoperative contrast-enhanced CT scan revealed a left temporal tumor A second surgery was therefore performed for initial tumor removal 2 months later Histopathological findings showed that the tumor was typical PXA with strong pleomorphism and xanthomatous changes and contained an ependymoma-like component in the center area However endothelial proliferation and mitosis were more remarkable compared to ordinary PXA The MIB-1 labeling index was 98 high From these findings the histopathological diagnosis was anaplastic PXA The patient underwent surgery to remove recurrent tumors 5 and 16 months later The patient died 36 months after the first onset and CT revealed glioblastoma-like

findings and cerebrospinal fluid dissemination This case report is the first case in which PXA presented with tumor bleeding onset Histopathological findings suggested anaplastic PXA from the first surgical specimens and PXA recurred many times We thus believe that the patient displayed primary anaplastic PXA rather than secondary anaplastic PXA that results in malignant transformation10202016 33unusual giomas

Ganglioglioma

bull WHO grade 1bull Initially described by

Perkins in 1926bull Benign well

differentiatedneuroepithelial tumors

bull Most commonly found intemporal lobe though canoccur anywhere in CNS

bull Commonly found inyoung patients withepilepsy

bull Most commonly notedtumor in temporal lobeepilepsy patients

bull Have been described inbrain steem spinal cordand cerebellum

bull Account for 1 ofprimary brain tumors

10202016 34unusual giomas

bull Male dominencebull Most common features

are seizures headachedizziness ataxiaprogressive weakness

bull Malignanttransformation is rare

bull Histology showsimmature mixture ofabnormal neural andglial elements

bull Macroscopically appearsolid or cystic

bull Cystic are welldelineatedfromsurrounding brain

bull Calcification is common

10202016 35unusual giomas

bull IHC staing shows reactivity for GFAP S-100 andvimentin

bull Neurons show synaptophysin class III betatubulin NFP chromogranin reactivity

10202016 36unusual giomas

Imaging

bull Highly variable imagingcharacters

bull No special typicalimaging criteria

bull Enhancement patternsare highly variable

bull Iso to hypointense onT1WI

bull Heterogenous andhyperintense on T2WI

bull Can contain solid cyticcalcified components

10202016 37unusual giomas

bull FDG PET scan showshypometabolism oreumetabolism in thesepatients

10202016 38unusual giomas

Management And Outcome

bull Gold standard is grosstotal resection

bull Seizure control aftersurgery is as high as80

bull Survival rates are highbull Event free survival of

low grade and highgrade gangiogioma are57 vs 15

bull The optimal surgicalresection depends uponthe eloquent areasinvolved in the vicinityof the tumor

bull The use ofchemotherapy is notwell established

bull Typically they shouldbe allowed for chemoand radiotherapy

10202016 39unusual giomas

bull The use of adjuvant therapy is warranted in thosecases which harbour residual lesions or features ofanaplasia

bull Potential harm to nearby brain areas preclude thejudicious use of radiation

10202016 40unusual giomas

bull Brain Tumor Res Treat 2014 Oct 2(2) 49ndash55

bull Treatment and Outcomes for Gangliogliomas A Single-Center Review of 16 Patients

bull Jye Young Song Jeong Hoon Kim Young Hyun Cho Chang Jin Kim and Eun Jung Lee

bull Ganglioglioma is a rare and slowly growing benign tumor We investigated the outcomes of patients who underwent different combination treatments

bull Methods

bull Between 1998 and 2012 16 patients including 11 men and 5 women with a median age of 125 years (range 25-65 years) were treated for intracranial gangliogliomas at our institution The median follow-up period was 57 years (range 48 days-156 years) Fifteen cases were included in the outcome assessment because one patient was lost to follow-up Complete resection was achieved in 8 (53) patients Six (40) patients underwent incomplete resection with or without adjuvant radiotherapy and one patient with a brainstem tumor underwent only stereotactic biopsy

bull Results

bull Gangliogliomas predominantly affected young (875) male patients and most frequently presented with seizures (64) Of eight patients who underwent complete resection seven did not show recurrence whereas only three of six with incomplete resection showed no recurrence Four patients with recurrence received salvage treatments (two repeat surgeries and two radiosurgeries) A tumor control rate of 93 (1415) was achieved at the last follow-up No recurrence or malignant changes were observed after a median follow-up of 12 and 45 years in four patients who received gamma knife (GK) radiosurgery as adjuvant and salvage treatment

bull Conclusion

bull Complete resection produced the best outcomes and incomplete resection followed by adjuvant or salvage treatments showed favorable outcomes In patients who are not eligible for complete resection because of tumor location or potential neurologic deficits following surgery GK radiosurgery should be considered for the treatment of residual or recurrent tumors

10202016 41unusual giomas

Papillary tumours of pineal

bull WHO grade II AND IIIbull Relatively new edition to WHObull They are equal in both sexesbull Reach medical attention due to headache mental

status changes and altered visionbull Contain eosinophilic cells with distinctive

borders with large pleomorphic nucleibull Positive for cytokeratin synaptophysin stain

variably for GFAP vimentin S-100

10202016 unusual giomas 42

Imaging

bull Generally found to be diffusely enhancinglesion of pineal region

bull Post 3rd ventricle with HCPbull Appear well demarcated from thalamus and

cerebellum

10202016 unusual giomas 43

Mangement

bull Standard treatment is excisionbull Grow slowly and donot transform to

malignant tumorsbull Local recurrence is however possiblebull Radiotherapy appears to provide good control

in local recurrence

10202016 unusual giomas 44

Choroid Glioma Of Third Ventricle

bull WHO Grade IIbull Ist recognised by WHO in

1998bull Arise in the anterior third

ventriclebull Commonly come to

attention after HCPbull Appera to contain in 3rd

ventricle and thought toarise from ventricularsurface

bull Composed of GFAP andvimentin positive cells inmucinous matrix

bull Nuclei are uniform insize and shape

bull Indolent growthbull May show EGF receptor

positivitybull Some suggets these tumor

arise from the lminaterminalis andventricular tanycytes

10202016 45unusual giomas

Imaging

bull Commonly fill 3rd

ventriclebull Diffusely enhance with

contrastbull Appear to infiltrate the

thalamusbull Large tumors may be

difficult to differentiatefrom suprasellar masses

10202016 46unusual giomas

10202016 47unusual giomas

bull Surgical management is limited by locationbull Access through the dialted ventricle and lamina

terminalis may be challangingbull Subtotal resection is thus commonbull Role of adjuvant radiotherapy is unknown

10202016 48unusual giomas

bull Acta Neurochir (Wien) 2008 Nov150(11)1183-91 doi 101007s00701-008-0014-6 Epub

2008 Oct 21

bull Chordoid glioma of the third ventricle

bull Vanhauwaert DJ1 Clement F Van Dorpe J Deruytter MJKGROUND

bull Chordoid glioma is a rare tumour (World Health Organisation grade II) originating from the third ventricle

with both glial and chordoid features It was first described by Brat in 1998 Since there is no detailed

information available on the outcome after surgery and adjuvant treatment we reviewed the literature

bull METHODS

bull A literature search through PUBMED revealed 50 cases of chordoid glioma Most reports

were found in pathology journals Information on the postoperative course was sometimes

very limited We reviewed the available literature and studied in detail the presenting

symptoms mortality and postoperative complications in relation to the extent of resective

surgery as well as the importance of adjuvant treatment

bull CONCLUSIONS

bull Mortality in the immediate postoperative period is 32 and is higher after gross total

resection as compared to subtotal resection Non-fatal postoperative complications are

hypothalamic disorders and mental alterations Gross total resection is the treatment of

choice since no recurrence has been reported after macroscopically complete resection

but this is often difficult because of the location and adherence to the hypothalamus The

role of postoperative radiotherapy is uncertain There is some indication that radiosurgery

with or without conventional irradiation is superior to conventional radiation alone Planned

subtotal resection followed by stereotactic radiosurgery can be a safe and effective

alternative in a patient in whom gross total resection is considered to be too risky

There is no report on the use of chemotherapy in the treatment of chordoid gliomas More

information about the optimal treatment strategy is needed and more reports are also needed10202016 49unusual giomas

bull Neurologist 2011 Jan17(1)52bull Chordoid glioma a case report and literature reviewbull Liu WP1 Cheng JX Yi XC Zhen HN Fei Z Li Q Zhang Xbull chordoid glioma is a rare tumor (World Health Organization grade II) with both

glial and chordoid features often located in the suprasellar region and anterior third ventricle It was first described by Brat in 1998 Because there is no detailed information available from the clinical perspective we reviewed the literature

bull METHODS

bull a literature search through PUBMED and CNKI revealed 64 cases of chordoid glioma Information on the clinical course was very limited We reviewed the literature and studied the pathologic and imaging features postoperative mortality and morbidity in relation to surgical extension and approaches and the importance of adjuvant treatment

bull CONCLUSIONS bull mortality in the immediate postoperative period is 28 and postoperative

morbidity is 60 which are statistically higher after gross total resection as compared with subtotal resection Translamina terminalis approach is considered to be the best approach The current study cannot document that patients have longer survival and higher quality of life after gross total resection than subtotal resection The role of postoperative radiotherapy is uncertain and there is no report on the use of chemotherapy More information about the optimal treatment strategy is needed

10202016 50unusual giomas

Conclusion

bull They are identified by their unique morphologicalfeatures

bull Many of these have indolent course and identified byepilepsy

bull Potentail diagnosis should be considered in youngpatients with seizures and cortically based lesions

bull Correct diagnosis requires experiencedneuropathologisrt

bull As more experienced is gained more knowledgetowards better understanding shall provide betteroutcomes in these patients

10202016 unusual giomas 51

10202016 52unusual giomas

Content supportndash youmann neurosurgery textbook

Astroblastoma

bull WHO grade 1bull Initially described by Bailey

and Bucy in 1930bull Has been a matter of debate

for similar features asanaplastic astrocytomaglioblastoma andgemistocytic astrocytomas

bull Commonly found in 1st

three decades of lifebull Female

preponderance(111)

bull Affects the hemispheresinvolving cortex subcortical andperiventricular regions

bull No infratentorail tumorhave been reported

bull Pts present with features ofraised ICP cortical deficitsseizures personalitychanges

10202016 14unusual giomas

bull Greatly variable naturalhistory

bull Have a slow andindolent course

bull Rarely show anaggressive outcome

bull Almost always definedby their grossappearance

bull Cut surface revealhomogenous soft pinkgrey substances

bull Cystic areas arefrequently encountered

bull The clusters of cellsform pseudorossette

10202016 15unusual giomas

bull Richly supplied byblood vessels

bull Nuclei of tumor cellsare away from bloodvessels and send fineprocesse to the vesselwall

bull High number of mitoticfigures can be found

bull Cellular atypia isfrequently noted

bull On IHC tumor cellsstain positive for GFAP

bull Positivity for vimentinNSE S-1OO EMA

bull These are positive foranti leu 7 antibodiesspecific for cells ofepithelial origin

bull Central necrosis can befound

10202016 16unusual giomas

Imaging

bull On CT the appearnacesvaries from poorlydefined hypodensetumor to welldemarcated and highlyenhancing masses

10202016 17unusual giomas

bull On MR image tumorsare hypointense towhite matter on T1

bull Hyperintense on T2bull Hyperintense on FLAIRbull Enhancement is seen

around the cystic centre

10202016 18unusual giomas

Management And Prognosis

bull Surgical resection is main treatmentbull Gross total resection can be easily achieved as

lesions are mostly in the cortical areasbull Therapeutic value is of radiation and

chemotherapy is uncertain as these are raretumors

bull Prediction of outcome is difficult as these remainindolent for a long time and may rapidlytransform to glioblastoma resulting in fataloutcomes

10202016 19unusual giomas

Pilomyxoid astrocytoma

bull Recently described tumorsbull Similarities with pilocytic

astrocytomasbull Until recently these were

grouped with PAbull These involve entire

neuraxisbull Mean age at preentation is

18 monthsbull Most are found in

hypothalamic ndash chiasmicregion

bull Pts present with features ofraised ICP

bull RAISED HEADCIRCUMFERENCE may beonly subtle sign in infants

bull Histologically consists ofmyxoid matrix with cells inloose fibrillary and myxoidbackground

bull Tumor cells formperivascular rosettes

10202016 20unusual giomas

bull Preferred sites includebull (1) optic nerve (lsquooptic

nerve gliomarsquo)bull (2) optic

chiasmhypothalamusbull (3) thalamus and basal

gangliabull (4) cerebral hemispherebull (5) cerebellum (lsquocerebellar

astrocytomarsquo) andbull (6) brainstem (dorsal

exophytic brainstemglioma)

bull Genetically chracterized bythe presence of NF1mutations BRAFduplications and theabsence of IDH1 mutation

10202016 21unusual giomas

Imaging

bull The huge chiasmatic-hypothalamic tumor revealslow signal intensity on aT1-weighted image (A)

bull Bright signal intensity on aT2-weighted image (B)

bull Gadolinium-enhancedbrain MR images showheterogeneously strongenhancement (C and D)

bull Leptomeningealdisseminations at thecervical spine levels

10202016 22unusual giomas

bull NO standard careprotocols have beendefined

bull As these are newlydefined and raretumors

bull Surgical resection isfavoured if locationfavours

bull Mean progression freesurvival is 25 months

bull Mean overall survival is60 months

bull Reduced survival isbecause of inability toachieve gross resection

10202016 23unusual giomas

bull The usual indications foradjuvant treatment(chemotherapy orradiation therapy)include tumor recurrenceafter initial completeresection or symptomatictumors

bull In addition adjuvanttreatment is generallyindicated for tumors withgrowth on follow-upimaging even in theabsence of symptoms

bull The role of chemotherapyis in evolution

bull Chemotherapy may beimplemented for the treatment ofinoperable or partially resectedgliomas

bull The decision to use postoperativeadjuvant therapy also depends ona lesions perceived malignanttendencies as the potentialbenefits of radiation therapy andchemotherapy must be weighedagainst their morbidity

bull The decision to use postoperativeadjuvant therapy also depends ona lesions perceived malignanttendencies as the potentialbenefits of radiation therapy andchemotherapy must be weighedagainst their morbidity

10202016 24unusual giomas

Pleomorphic xanthoastrocytoma

bull First described by Kepsand colleuges in 1979

bull Represents lt1 of allastrocytomas

bull May be found alongentire neuraxis mostlysupratentorial( temporallobe)

bull Cerebellum and soinalcord are the other sites

bull Pts present in 2nd and 3rd

decadesbull Median age at 14 yearsbull Both sexes are affected

equallybull 70-80 pts present with

seizures and headachefocal neuro deficits

bull Rarely tumor mayhemmorrhage

10202016 25unusual giomas

bull Supratentorail are mostoften cortical and meningesbased

bull Chronic seizuresbull Grossely these are firm

tumors variable in colorrelatively avascular

bull Typically invade the piaarachnoid space

bull 13 pts have involvementof all the three meninges

bull Cyst fluid is xanthochromic

bull Very specific microscpoiccriteria for diagnosis are

1 Pleomorphic andxanthomatous astrocytes

2 Perivascular lymphocytes3 Non infilterating histology4 Reticulin stainingbull Pleomorphism can be as

vast as glioblastoma orsarcomatous tumors

10202016 26unusual giomas

bull IHC Staining revealsspindle cells and roundpleomorphic cellspositive for S-100 andGFAP

bull Staining forsynaptophysin and NSEmay be also present

bull Some workers havereported them to be asubtype of ganglioglioma

bull Association between theNeurofibromatosis Sturge Weber Syndrome

bull Loss of chr 9 and gain ofchromosome 7 have beenfound

10202016 27unusual giomas

Imaging

bull CTbull PXAs are typically hypo

or isodense and may bewell or poorlydemarcated usually withno or little surroundingoedema

bull Calcification is rarebull Due to its superficial

location it may causescalloping of theoverlying bone

10202016 28unusual giomas

bull MRIbull T1

ndash solid component iso tohypointense cf grey matter

ndash cystic component low signalndash leptomeningeal involvement

seen in over 70 of casesbull T1 C+ (Gd)

ndash solid component usuallyenhances vividly

bull T2ndash solid component iso to

hyperintense cf grey matterndash cystic component high signalndash on T2 FLAIR sequence cystic

areas show hyperintensityrelative to CSF due to higherprotein contents

ndash little surrounding vasogenicoedema

10202016 29unusual giomas

Management amp Outcome

bull Quite rare tumorsbull Surgical excision appears to

be best favoured modalitybull Actuarial survival trates are

91 at 5 years and 82 at10 years

bull Median survival 18 years

bull Total resection should beattempted in every case asfar as possible

bull Resection of cyst lining isnot indicated

bull Data regarding the use ofadjuvant therapy at thistime are limited to few casereports only

bull Pts who had incompleteresection high mitoticindex necrosis should bereserved for strict vigilanceand CEMRI on follow ups

10202016 30unusual giomas

bull At progression repeatresection be attempted

bull Proression should betaken as aggressivetumor type

bull Malignanttransformation maynecessitate adjuvanttherapy

10202016 31unusual giomas

bull Br J Neurosurg 2013 Dec27(6)759-64 doi 103109026886972013776666 Epub 2013 Mar 20

bull Pleomorphic xanthoastrocytoma long-term results of surgical treatment and analysis of prognosticfactors

bull Gallo P1 Cecchi PC Locatelli F Rizzo P Ghimenton C Gerosa M Pinna G

bull BACKGROUND

bull Pleomorphic Xanthoastrocytoma (PXA) is a rare brain tumour most commonly affecting children andyoung adults To date only few data regarding the long-term follow-up of these patients after surgery are

available The aim of this study is to describe our single-institution experience in the surgicalmanagement of this particular glioma over a period of over 18 years

bull METHODS

bull We performed a retrospective review of all cases of PXA (40 patients) operated upon at theDepartment of Neurosurgery of Verona Italy between 1990 and 2008 The impact of clinical radiologicalsurgical and histological factors on overall survival (OS) and progression-free survival (PFS) was analysed bymeans of univariate and multivariate models

bull FINDINGS

bull We achieved a gross total resection (GTR) in 65 of patients Histological diagnosis was of grade II in 80anaplastic features were present in the remaining 20 Adjuvant treatment radiotherapy or chemo-

radiotherapy was administered in 40 of the cases Median follow-up was 74 months OS at 5- and10 years was 7632 and 6824 respectively PFS at 5- and 10 years was 71 and 58respectively In the multivariate model histological grade extent of resection and age at diagnosis (le 30years vs gt 30 years) were the only independent prognostic factors for both OS and PFS

bull CONCLUSIONS

bull Our retrospective long-term study confirms the relatively favourable prognosis associatedwith PXA Young patients with a low-grade tumour (WHO grade II) who underwent GTR carrythe longest OS and PFS

10202016 32unusual giomas

bull Brain Tumor Pathol 2006 Apr23(1)55-63

bull A case of anaplastic pleomorphic xanthoastrocytoma presenting with tumor bleeding and cerebrospinal fluid dissemination

bull Asano K1 Miyamoto S Kubo O Kikkukawa T Yagihashi A Ohkuma H

bull Pleomorphic xanthoastrocytoma (PXA) has been considered an astrocytic tumorwith a relatively favorable prognosis However PXA cases having several recurrent patterns with poor prognosis have been reported in recent years and a new concept of anaplastic PXA has been proposed The present case was a 59-year-old woman who presented with tumor bleeding onset and cerebrospinal fluid dissemination The patient had sudden-onset right hemiparesis aphasia and consciousness disturbance and was admitted to a local area hospital After emergency surgery had removed the hematoma postoperative contrast-enhanced CT scan revealed a left temporal tumor A second surgery was therefore performed for initial tumor removal 2 months later Histopathological findings showed that the tumor was typical PXA with strong pleomorphism and xanthomatous changes and contained an ependymoma-like component in the center area However endothelial proliferation and mitosis were more remarkable compared to ordinary PXA The MIB-1 labeling index was 98 high From these findings the histopathological diagnosis was anaplastic PXA The patient underwent surgery to remove recurrent tumors 5 and 16 months later The patient died 36 months after the first onset and CT revealed glioblastoma-like

findings and cerebrospinal fluid dissemination This case report is the first case in which PXA presented with tumor bleeding onset Histopathological findings suggested anaplastic PXA from the first surgical specimens and PXA recurred many times We thus believe that the patient displayed primary anaplastic PXA rather than secondary anaplastic PXA that results in malignant transformation10202016 33unusual giomas

Ganglioglioma

bull WHO grade 1bull Initially described by

Perkins in 1926bull Benign well

differentiatedneuroepithelial tumors

bull Most commonly found intemporal lobe though canoccur anywhere in CNS

bull Commonly found inyoung patients withepilepsy

bull Most commonly notedtumor in temporal lobeepilepsy patients

bull Have been described inbrain steem spinal cordand cerebellum

bull Account for 1 ofprimary brain tumors

10202016 34unusual giomas

bull Male dominencebull Most common features

are seizures headachedizziness ataxiaprogressive weakness

bull Malignanttransformation is rare

bull Histology showsimmature mixture ofabnormal neural andglial elements

bull Macroscopically appearsolid or cystic

bull Cystic are welldelineatedfromsurrounding brain

bull Calcification is common

10202016 35unusual giomas

bull IHC staing shows reactivity for GFAP S-100 andvimentin

bull Neurons show synaptophysin class III betatubulin NFP chromogranin reactivity

10202016 36unusual giomas

Imaging

bull Highly variable imagingcharacters

bull No special typicalimaging criteria

bull Enhancement patternsare highly variable

bull Iso to hypointense onT1WI

bull Heterogenous andhyperintense on T2WI

bull Can contain solid cyticcalcified components

10202016 37unusual giomas

bull FDG PET scan showshypometabolism oreumetabolism in thesepatients

10202016 38unusual giomas

Management And Outcome

bull Gold standard is grosstotal resection

bull Seizure control aftersurgery is as high as80

bull Survival rates are highbull Event free survival of

low grade and highgrade gangiogioma are57 vs 15

bull The optimal surgicalresection depends uponthe eloquent areasinvolved in the vicinityof the tumor

bull The use ofchemotherapy is notwell established

bull Typically they shouldbe allowed for chemoand radiotherapy

10202016 39unusual giomas

bull The use of adjuvant therapy is warranted in thosecases which harbour residual lesions or features ofanaplasia

bull Potential harm to nearby brain areas preclude thejudicious use of radiation

10202016 40unusual giomas

bull Brain Tumor Res Treat 2014 Oct 2(2) 49ndash55

bull Treatment and Outcomes for Gangliogliomas A Single-Center Review of 16 Patients

bull Jye Young Song Jeong Hoon Kim Young Hyun Cho Chang Jin Kim and Eun Jung Lee

bull Ganglioglioma is a rare and slowly growing benign tumor We investigated the outcomes of patients who underwent different combination treatments

bull Methods

bull Between 1998 and 2012 16 patients including 11 men and 5 women with a median age of 125 years (range 25-65 years) were treated for intracranial gangliogliomas at our institution The median follow-up period was 57 years (range 48 days-156 years) Fifteen cases were included in the outcome assessment because one patient was lost to follow-up Complete resection was achieved in 8 (53) patients Six (40) patients underwent incomplete resection with or without adjuvant radiotherapy and one patient with a brainstem tumor underwent only stereotactic biopsy

bull Results

bull Gangliogliomas predominantly affected young (875) male patients and most frequently presented with seizures (64) Of eight patients who underwent complete resection seven did not show recurrence whereas only three of six with incomplete resection showed no recurrence Four patients with recurrence received salvage treatments (two repeat surgeries and two radiosurgeries) A tumor control rate of 93 (1415) was achieved at the last follow-up No recurrence or malignant changes were observed after a median follow-up of 12 and 45 years in four patients who received gamma knife (GK) radiosurgery as adjuvant and salvage treatment

bull Conclusion

bull Complete resection produced the best outcomes and incomplete resection followed by adjuvant or salvage treatments showed favorable outcomes In patients who are not eligible for complete resection because of tumor location or potential neurologic deficits following surgery GK radiosurgery should be considered for the treatment of residual or recurrent tumors

10202016 41unusual giomas

Papillary tumours of pineal

bull WHO grade II AND IIIbull Relatively new edition to WHObull They are equal in both sexesbull Reach medical attention due to headache mental

status changes and altered visionbull Contain eosinophilic cells with distinctive

borders with large pleomorphic nucleibull Positive for cytokeratin synaptophysin stain

variably for GFAP vimentin S-100

10202016 unusual giomas 42

Imaging

bull Generally found to be diffusely enhancinglesion of pineal region

bull Post 3rd ventricle with HCPbull Appear well demarcated from thalamus and

cerebellum

10202016 unusual giomas 43

Mangement

bull Standard treatment is excisionbull Grow slowly and donot transform to

malignant tumorsbull Local recurrence is however possiblebull Radiotherapy appears to provide good control

in local recurrence

10202016 unusual giomas 44

Choroid Glioma Of Third Ventricle

bull WHO Grade IIbull Ist recognised by WHO in

1998bull Arise in the anterior third

ventriclebull Commonly come to

attention after HCPbull Appera to contain in 3rd

ventricle and thought toarise from ventricularsurface

bull Composed of GFAP andvimentin positive cells inmucinous matrix

bull Nuclei are uniform insize and shape

bull Indolent growthbull May show EGF receptor

positivitybull Some suggets these tumor

arise from the lminaterminalis andventricular tanycytes

10202016 45unusual giomas

Imaging

bull Commonly fill 3rd

ventriclebull Diffusely enhance with

contrastbull Appear to infiltrate the

thalamusbull Large tumors may be

difficult to differentiatefrom suprasellar masses

10202016 46unusual giomas

10202016 47unusual giomas

bull Surgical management is limited by locationbull Access through the dialted ventricle and lamina

terminalis may be challangingbull Subtotal resection is thus commonbull Role of adjuvant radiotherapy is unknown

10202016 48unusual giomas

bull Acta Neurochir (Wien) 2008 Nov150(11)1183-91 doi 101007s00701-008-0014-6 Epub

2008 Oct 21

bull Chordoid glioma of the third ventricle

bull Vanhauwaert DJ1 Clement F Van Dorpe J Deruytter MJKGROUND

bull Chordoid glioma is a rare tumour (World Health Organisation grade II) originating from the third ventricle

with both glial and chordoid features It was first described by Brat in 1998 Since there is no detailed

information available on the outcome after surgery and adjuvant treatment we reviewed the literature

bull METHODS

bull A literature search through PUBMED revealed 50 cases of chordoid glioma Most reports

were found in pathology journals Information on the postoperative course was sometimes

very limited We reviewed the available literature and studied in detail the presenting

symptoms mortality and postoperative complications in relation to the extent of resective

surgery as well as the importance of adjuvant treatment

bull CONCLUSIONS

bull Mortality in the immediate postoperative period is 32 and is higher after gross total

resection as compared to subtotal resection Non-fatal postoperative complications are

hypothalamic disorders and mental alterations Gross total resection is the treatment of

choice since no recurrence has been reported after macroscopically complete resection

but this is often difficult because of the location and adherence to the hypothalamus The

role of postoperative radiotherapy is uncertain There is some indication that radiosurgery

with or without conventional irradiation is superior to conventional radiation alone Planned

subtotal resection followed by stereotactic radiosurgery can be a safe and effective

alternative in a patient in whom gross total resection is considered to be too risky

There is no report on the use of chemotherapy in the treatment of chordoid gliomas More

information about the optimal treatment strategy is needed and more reports are also needed10202016 49unusual giomas

bull Neurologist 2011 Jan17(1)52bull Chordoid glioma a case report and literature reviewbull Liu WP1 Cheng JX Yi XC Zhen HN Fei Z Li Q Zhang Xbull chordoid glioma is a rare tumor (World Health Organization grade II) with both

glial and chordoid features often located in the suprasellar region and anterior third ventricle It was first described by Brat in 1998 Because there is no detailed information available from the clinical perspective we reviewed the literature

bull METHODS

bull a literature search through PUBMED and CNKI revealed 64 cases of chordoid glioma Information on the clinical course was very limited We reviewed the literature and studied the pathologic and imaging features postoperative mortality and morbidity in relation to surgical extension and approaches and the importance of adjuvant treatment

bull CONCLUSIONS bull mortality in the immediate postoperative period is 28 and postoperative

morbidity is 60 which are statistically higher after gross total resection as compared with subtotal resection Translamina terminalis approach is considered to be the best approach The current study cannot document that patients have longer survival and higher quality of life after gross total resection than subtotal resection The role of postoperative radiotherapy is uncertain and there is no report on the use of chemotherapy More information about the optimal treatment strategy is needed

10202016 50unusual giomas

Conclusion

bull They are identified by their unique morphologicalfeatures

bull Many of these have indolent course and identified byepilepsy

bull Potentail diagnosis should be considered in youngpatients with seizures and cortically based lesions

bull Correct diagnosis requires experiencedneuropathologisrt

bull As more experienced is gained more knowledgetowards better understanding shall provide betteroutcomes in these patients

10202016 unusual giomas 51

10202016 52unusual giomas

Content supportndash youmann neurosurgery textbook

bull Greatly variable naturalhistory

bull Have a slow andindolent course

bull Rarely show anaggressive outcome

bull Almost always definedby their grossappearance

bull Cut surface revealhomogenous soft pinkgrey substances

bull Cystic areas arefrequently encountered

bull The clusters of cellsform pseudorossette

10202016 15unusual giomas

bull Richly supplied byblood vessels

bull Nuclei of tumor cellsare away from bloodvessels and send fineprocesse to the vesselwall

bull High number of mitoticfigures can be found

bull Cellular atypia isfrequently noted

bull On IHC tumor cellsstain positive for GFAP

bull Positivity for vimentinNSE S-1OO EMA

bull These are positive foranti leu 7 antibodiesspecific for cells ofepithelial origin

bull Central necrosis can befound

10202016 16unusual giomas

Imaging

bull On CT the appearnacesvaries from poorlydefined hypodensetumor to welldemarcated and highlyenhancing masses

10202016 17unusual giomas

bull On MR image tumorsare hypointense towhite matter on T1

bull Hyperintense on T2bull Hyperintense on FLAIRbull Enhancement is seen

around the cystic centre

10202016 18unusual giomas

Management And Prognosis

bull Surgical resection is main treatmentbull Gross total resection can be easily achieved as

lesions are mostly in the cortical areasbull Therapeutic value is of radiation and

chemotherapy is uncertain as these are raretumors

bull Prediction of outcome is difficult as these remainindolent for a long time and may rapidlytransform to glioblastoma resulting in fataloutcomes

10202016 19unusual giomas

Pilomyxoid astrocytoma

bull Recently described tumorsbull Similarities with pilocytic

astrocytomasbull Until recently these were

grouped with PAbull These involve entire

neuraxisbull Mean age at preentation is

18 monthsbull Most are found in

hypothalamic ndash chiasmicregion

bull Pts present with features ofraised ICP

bull RAISED HEADCIRCUMFERENCE may beonly subtle sign in infants

bull Histologically consists ofmyxoid matrix with cells inloose fibrillary and myxoidbackground

bull Tumor cells formperivascular rosettes

10202016 20unusual giomas

bull Preferred sites includebull (1) optic nerve (lsquooptic

nerve gliomarsquo)bull (2) optic

chiasmhypothalamusbull (3) thalamus and basal

gangliabull (4) cerebral hemispherebull (5) cerebellum (lsquocerebellar

astrocytomarsquo) andbull (6) brainstem (dorsal

exophytic brainstemglioma)

bull Genetically chracterized bythe presence of NF1mutations BRAFduplications and theabsence of IDH1 mutation

10202016 21unusual giomas

Imaging

bull The huge chiasmatic-hypothalamic tumor revealslow signal intensity on aT1-weighted image (A)

bull Bright signal intensity on aT2-weighted image (B)

bull Gadolinium-enhancedbrain MR images showheterogeneously strongenhancement (C and D)

bull Leptomeningealdisseminations at thecervical spine levels

10202016 22unusual giomas

bull NO standard careprotocols have beendefined

bull As these are newlydefined and raretumors

bull Surgical resection isfavoured if locationfavours

bull Mean progression freesurvival is 25 months

bull Mean overall survival is60 months

bull Reduced survival isbecause of inability toachieve gross resection

10202016 23unusual giomas

bull The usual indications foradjuvant treatment(chemotherapy orradiation therapy)include tumor recurrenceafter initial completeresection or symptomatictumors

bull In addition adjuvanttreatment is generallyindicated for tumors withgrowth on follow-upimaging even in theabsence of symptoms

bull The role of chemotherapyis in evolution

bull Chemotherapy may beimplemented for the treatment ofinoperable or partially resectedgliomas

bull The decision to use postoperativeadjuvant therapy also depends ona lesions perceived malignanttendencies as the potentialbenefits of radiation therapy andchemotherapy must be weighedagainst their morbidity

bull The decision to use postoperativeadjuvant therapy also depends ona lesions perceived malignanttendencies as the potentialbenefits of radiation therapy andchemotherapy must be weighedagainst their morbidity

10202016 24unusual giomas

Pleomorphic xanthoastrocytoma

bull First described by Kepsand colleuges in 1979

bull Represents lt1 of allastrocytomas

bull May be found alongentire neuraxis mostlysupratentorial( temporallobe)

bull Cerebellum and soinalcord are the other sites

bull Pts present in 2nd and 3rd

decadesbull Median age at 14 yearsbull Both sexes are affected

equallybull 70-80 pts present with

seizures and headachefocal neuro deficits

bull Rarely tumor mayhemmorrhage

10202016 25unusual giomas

bull Supratentorail are mostoften cortical and meningesbased

bull Chronic seizuresbull Grossely these are firm

tumors variable in colorrelatively avascular

bull Typically invade the piaarachnoid space

bull 13 pts have involvementof all the three meninges

bull Cyst fluid is xanthochromic

bull Very specific microscpoiccriteria for diagnosis are

1 Pleomorphic andxanthomatous astrocytes

2 Perivascular lymphocytes3 Non infilterating histology4 Reticulin stainingbull Pleomorphism can be as

vast as glioblastoma orsarcomatous tumors

10202016 26unusual giomas

bull IHC Staining revealsspindle cells and roundpleomorphic cellspositive for S-100 andGFAP

bull Staining forsynaptophysin and NSEmay be also present

bull Some workers havereported them to be asubtype of ganglioglioma

bull Association between theNeurofibromatosis Sturge Weber Syndrome

bull Loss of chr 9 and gain ofchromosome 7 have beenfound

10202016 27unusual giomas

Imaging

bull CTbull PXAs are typically hypo

or isodense and may bewell or poorlydemarcated usually withno or little surroundingoedema

bull Calcification is rarebull Due to its superficial

location it may causescalloping of theoverlying bone

10202016 28unusual giomas

bull MRIbull T1

ndash solid component iso tohypointense cf grey matter

ndash cystic component low signalndash leptomeningeal involvement

seen in over 70 of casesbull T1 C+ (Gd)

ndash solid component usuallyenhances vividly

bull T2ndash solid component iso to

hyperintense cf grey matterndash cystic component high signalndash on T2 FLAIR sequence cystic

areas show hyperintensityrelative to CSF due to higherprotein contents

ndash little surrounding vasogenicoedema

10202016 29unusual giomas

Management amp Outcome

bull Quite rare tumorsbull Surgical excision appears to

be best favoured modalitybull Actuarial survival trates are

91 at 5 years and 82 at10 years

bull Median survival 18 years

bull Total resection should beattempted in every case asfar as possible

bull Resection of cyst lining isnot indicated

bull Data regarding the use ofadjuvant therapy at thistime are limited to few casereports only

bull Pts who had incompleteresection high mitoticindex necrosis should bereserved for strict vigilanceand CEMRI on follow ups

10202016 30unusual giomas

bull At progression repeatresection be attempted

bull Proression should betaken as aggressivetumor type

bull Malignanttransformation maynecessitate adjuvanttherapy

10202016 31unusual giomas

bull Br J Neurosurg 2013 Dec27(6)759-64 doi 103109026886972013776666 Epub 2013 Mar 20

bull Pleomorphic xanthoastrocytoma long-term results of surgical treatment and analysis of prognosticfactors

bull Gallo P1 Cecchi PC Locatelli F Rizzo P Ghimenton C Gerosa M Pinna G

bull BACKGROUND

bull Pleomorphic Xanthoastrocytoma (PXA) is a rare brain tumour most commonly affecting children andyoung adults To date only few data regarding the long-term follow-up of these patients after surgery are

available The aim of this study is to describe our single-institution experience in the surgicalmanagement of this particular glioma over a period of over 18 years

bull METHODS

bull We performed a retrospective review of all cases of PXA (40 patients) operated upon at theDepartment of Neurosurgery of Verona Italy between 1990 and 2008 The impact of clinical radiologicalsurgical and histological factors on overall survival (OS) and progression-free survival (PFS) was analysed bymeans of univariate and multivariate models

bull FINDINGS

bull We achieved a gross total resection (GTR) in 65 of patients Histological diagnosis was of grade II in 80anaplastic features were present in the remaining 20 Adjuvant treatment radiotherapy or chemo-

radiotherapy was administered in 40 of the cases Median follow-up was 74 months OS at 5- and10 years was 7632 and 6824 respectively PFS at 5- and 10 years was 71 and 58respectively In the multivariate model histological grade extent of resection and age at diagnosis (le 30years vs gt 30 years) were the only independent prognostic factors for both OS and PFS

bull CONCLUSIONS

bull Our retrospective long-term study confirms the relatively favourable prognosis associatedwith PXA Young patients with a low-grade tumour (WHO grade II) who underwent GTR carrythe longest OS and PFS

10202016 32unusual giomas

bull Brain Tumor Pathol 2006 Apr23(1)55-63

bull A case of anaplastic pleomorphic xanthoastrocytoma presenting with tumor bleeding and cerebrospinal fluid dissemination

bull Asano K1 Miyamoto S Kubo O Kikkukawa T Yagihashi A Ohkuma H

bull Pleomorphic xanthoastrocytoma (PXA) has been considered an astrocytic tumorwith a relatively favorable prognosis However PXA cases having several recurrent patterns with poor prognosis have been reported in recent years and a new concept of anaplastic PXA has been proposed The present case was a 59-year-old woman who presented with tumor bleeding onset and cerebrospinal fluid dissemination The patient had sudden-onset right hemiparesis aphasia and consciousness disturbance and was admitted to a local area hospital After emergency surgery had removed the hematoma postoperative contrast-enhanced CT scan revealed a left temporal tumor A second surgery was therefore performed for initial tumor removal 2 months later Histopathological findings showed that the tumor was typical PXA with strong pleomorphism and xanthomatous changes and contained an ependymoma-like component in the center area However endothelial proliferation and mitosis were more remarkable compared to ordinary PXA The MIB-1 labeling index was 98 high From these findings the histopathological diagnosis was anaplastic PXA The patient underwent surgery to remove recurrent tumors 5 and 16 months later The patient died 36 months after the first onset and CT revealed glioblastoma-like

findings and cerebrospinal fluid dissemination This case report is the first case in which PXA presented with tumor bleeding onset Histopathological findings suggested anaplastic PXA from the first surgical specimens and PXA recurred many times We thus believe that the patient displayed primary anaplastic PXA rather than secondary anaplastic PXA that results in malignant transformation10202016 33unusual giomas

Ganglioglioma

bull WHO grade 1bull Initially described by

Perkins in 1926bull Benign well

differentiatedneuroepithelial tumors

bull Most commonly found intemporal lobe though canoccur anywhere in CNS

bull Commonly found inyoung patients withepilepsy

bull Most commonly notedtumor in temporal lobeepilepsy patients

bull Have been described inbrain steem spinal cordand cerebellum

bull Account for 1 ofprimary brain tumors

10202016 34unusual giomas

bull Male dominencebull Most common features

are seizures headachedizziness ataxiaprogressive weakness

bull Malignanttransformation is rare

bull Histology showsimmature mixture ofabnormal neural andglial elements

bull Macroscopically appearsolid or cystic

bull Cystic are welldelineatedfromsurrounding brain

bull Calcification is common

10202016 35unusual giomas

bull IHC staing shows reactivity for GFAP S-100 andvimentin

bull Neurons show synaptophysin class III betatubulin NFP chromogranin reactivity

10202016 36unusual giomas

Imaging

bull Highly variable imagingcharacters

bull No special typicalimaging criteria

bull Enhancement patternsare highly variable

bull Iso to hypointense onT1WI

bull Heterogenous andhyperintense on T2WI

bull Can contain solid cyticcalcified components

10202016 37unusual giomas

bull FDG PET scan showshypometabolism oreumetabolism in thesepatients

10202016 38unusual giomas

Management And Outcome

bull Gold standard is grosstotal resection

bull Seizure control aftersurgery is as high as80

bull Survival rates are highbull Event free survival of

low grade and highgrade gangiogioma are57 vs 15

bull The optimal surgicalresection depends uponthe eloquent areasinvolved in the vicinityof the tumor

bull The use ofchemotherapy is notwell established

bull Typically they shouldbe allowed for chemoand radiotherapy

10202016 39unusual giomas

bull The use of adjuvant therapy is warranted in thosecases which harbour residual lesions or features ofanaplasia

bull Potential harm to nearby brain areas preclude thejudicious use of radiation

10202016 40unusual giomas

bull Brain Tumor Res Treat 2014 Oct 2(2) 49ndash55

bull Treatment and Outcomes for Gangliogliomas A Single-Center Review of 16 Patients

bull Jye Young Song Jeong Hoon Kim Young Hyun Cho Chang Jin Kim and Eun Jung Lee

bull Ganglioglioma is a rare and slowly growing benign tumor We investigated the outcomes of patients who underwent different combination treatments

bull Methods

bull Between 1998 and 2012 16 patients including 11 men and 5 women with a median age of 125 years (range 25-65 years) were treated for intracranial gangliogliomas at our institution The median follow-up period was 57 years (range 48 days-156 years) Fifteen cases were included in the outcome assessment because one patient was lost to follow-up Complete resection was achieved in 8 (53) patients Six (40) patients underwent incomplete resection with or without adjuvant radiotherapy and one patient with a brainstem tumor underwent only stereotactic biopsy

bull Results

bull Gangliogliomas predominantly affected young (875) male patients and most frequently presented with seizures (64) Of eight patients who underwent complete resection seven did not show recurrence whereas only three of six with incomplete resection showed no recurrence Four patients with recurrence received salvage treatments (two repeat surgeries and two radiosurgeries) A tumor control rate of 93 (1415) was achieved at the last follow-up No recurrence or malignant changes were observed after a median follow-up of 12 and 45 years in four patients who received gamma knife (GK) radiosurgery as adjuvant and salvage treatment

bull Conclusion

bull Complete resection produced the best outcomes and incomplete resection followed by adjuvant or salvage treatments showed favorable outcomes In patients who are not eligible for complete resection because of tumor location or potential neurologic deficits following surgery GK radiosurgery should be considered for the treatment of residual or recurrent tumors

10202016 41unusual giomas

Papillary tumours of pineal

bull WHO grade II AND IIIbull Relatively new edition to WHObull They are equal in both sexesbull Reach medical attention due to headache mental

status changes and altered visionbull Contain eosinophilic cells with distinctive

borders with large pleomorphic nucleibull Positive for cytokeratin synaptophysin stain

variably for GFAP vimentin S-100

10202016 unusual giomas 42

Imaging

bull Generally found to be diffusely enhancinglesion of pineal region

bull Post 3rd ventricle with HCPbull Appear well demarcated from thalamus and

cerebellum

10202016 unusual giomas 43

Mangement

bull Standard treatment is excisionbull Grow slowly and donot transform to

malignant tumorsbull Local recurrence is however possiblebull Radiotherapy appears to provide good control

in local recurrence

10202016 unusual giomas 44

Choroid Glioma Of Third Ventricle

bull WHO Grade IIbull Ist recognised by WHO in

1998bull Arise in the anterior third

ventriclebull Commonly come to

attention after HCPbull Appera to contain in 3rd

ventricle and thought toarise from ventricularsurface

bull Composed of GFAP andvimentin positive cells inmucinous matrix

bull Nuclei are uniform insize and shape

bull Indolent growthbull May show EGF receptor

positivitybull Some suggets these tumor

arise from the lminaterminalis andventricular tanycytes

10202016 45unusual giomas

Imaging

bull Commonly fill 3rd

ventriclebull Diffusely enhance with

contrastbull Appear to infiltrate the

thalamusbull Large tumors may be

difficult to differentiatefrom suprasellar masses

10202016 46unusual giomas

10202016 47unusual giomas

bull Surgical management is limited by locationbull Access through the dialted ventricle and lamina

terminalis may be challangingbull Subtotal resection is thus commonbull Role of adjuvant radiotherapy is unknown

10202016 48unusual giomas

bull Acta Neurochir (Wien) 2008 Nov150(11)1183-91 doi 101007s00701-008-0014-6 Epub

2008 Oct 21

bull Chordoid glioma of the third ventricle

bull Vanhauwaert DJ1 Clement F Van Dorpe J Deruytter MJKGROUND

bull Chordoid glioma is a rare tumour (World Health Organisation grade II) originating from the third ventricle

with both glial and chordoid features It was first described by Brat in 1998 Since there is no detailed

information available on the outcome after surgery and adjuvant treatment we reviewed the literature

bull METHODS

bull A literature search through PUBMED revealed 50 cases of chordoid glioma Most reports

were found in pathology journals Information on the postoperative course was sometimes

very limited We reviewed the available literature and studied in detail the presenting

symptoms mortality and postoperative complications in relation to the extent of resective

surgery as well as the importance of adjuvant treatment

bull CONCLUSIONS

bull Mortality in the immediate postoperative period is 32 and is higher after gross total

resection as compared to subtotal resection Non-fatal postoperative complications are

hypothalamic disorders and mental alterations Gross total resection is the treatment of

choice since no recurrence has been reported after macroscopically complete resection

but this is often difficult because of the location and adherence to the hypothalamus The

role of postoperative radiotherapy is uncertain There is some indication that radiosurgery

with or without conventional irradiation is superior to conventional radiation alone Planned

subtotal resection followed by stereotactic radiosurgery can be a safe and effective

alternative in a patient in whom gross total resection is considered to be too risky

There is no report on the use of chemotherapy in the treatment of chordoid gliomas More

information about the optimal treatment strategy is needed and more reports are also needed10202016 49unusual giomas

bull Neurologist 2011 Jan17(1)52bull Chordoid glioma a case report and literature reviewbull Liu WP1 Cheng JX Yi XC Zhen HN Fei Z Li Q Zhang Xbull chordoid glioma is a rare tumor (World Health Organization grade II) with both

glial and chordoid features often located in the suprasellar region and anterior third ventricle It was first described by Brat in 1998 Because there is no detailed information available from the clinical perspective we reviewed the literature

bull METHODS

bull a literature search through PUBMED and CNKI revealed 64 cases of chordoid glioma Information on the clinical course was very limited We reviewed the literature and studied the pathologic and imaging features postoperative mortality and morbidity in relation to surgical extension and approaches and the importance of adjuvant treatment

bull CONCLUSIONS bull mortality in the immediate postoperative period is 28 and postoperative

morbidity is 60 which are statistically higher after gross total resection as compared with subtotal resection Translamina terminalis approach is considered to be the best approach The current study cannot document that patients have longer survival and higher quality of life after gross total resection than subtotal resection The role of postoperative radiotherapy is uncertain and there is no report on the use of chemotherapy More information about the optimal treatment strategy is needed

10202016 50unusual giomas

Conclusion

bull They are identified by their unique morphologicalfeatures

bull Many of these have indolent course and identified byepilepsy

bull Potentail diagnosis should be considered in youngpatients with seizures and cortically based lesions

bull Correct diagnosis requires experiencedneuropathologisrt

bull As more experienced is gained more knowledgetowards better understanding shall provide betteroutcomes in these patients

10202016 unusual giomas 51

10202016 52unusual giomas

Content supportndash youmann neurosurgery textbook

bull Richly supplied byblood vessels

bull Nuclei of tumor cellsare away from bloodvessels and send fineprocesse to the vesselwall

bull High number of mitoticfigures can be found

bull Cellular atypia isfrequently noted

bull On IHC tumor cellsstain positive for GFAP

bull Positivity for vimentinNSE S-1OO EMA

bull These are positive foranti leu 7 antibodiesspecific for cells ofepithelial origin

bull Central necrosis can befound

10202016 16unusual giomas

Imaging

bull On CT the appearnacesvaries from poorlydefined hypodensetumor to welldemarcated and highlyenhancing masses

10202016 17unusual giomas

bull On MR image tumorsare hypointense towhite matter on T1

bull Hyperintense on T2bull Hyperintense on FLAIRbull Enhancement is seen

around the cystic centre

10202016 18unusual giomas

Management And Prognosis

bull Surgical resection is main treatmentbull Gross total resection can be easily achieved as

lesions are mostly in the cortical areasbull Therapeutic value is of radiation and

chemotherapy is uncertain as these are raretumors

bull Prediction of outcome is difficult as these remainindolent for a long time and may rapidlytransform to glioblastoma resulting in fataloutcomes

10202016 19unusual giomas

Pilomyxoid astrocytoma

bull Recently described tumorsbull Similarities with pilocytic

astrocytomasbull Until recently these were

grouped with PAbull These involve entire

neuraxisbull Mean age at preentation is

18 monthsbull Most are found in

hypothalamic ndash chiasmicregion

bull Pts present with features ofraised ICP

bull RAISED HEADCIRCUMFERENCE may beonly subtle sign in infants

bull Histologically consists ofmyxoid matrix with cells inloose fibrillary and myxoidbackground

bull Tumor cells formperivascular rosettes

10202016 20unusual giomas

bull Preferred sites includebull (1) optic nerve (lsquooptic

nerve gliomarsquo)bull (2) optic

chiasmhypothalamusbull (3) thalamus and basal

gangliabull (4) cerebral hemispherebull (5) cerebellum (lsquocerebellar

astrocytomarsquo) andbull (6) brainstem (dorsal

exophytic brainstemglioma)

bull Genetically chracterized bythe presence of NF1mutations BRAFduplications and theabsence of IDH1 mutation

10202016 21unusual giomas

Imaging

bull The huge chiasmatic-hypothalamic tumor revealslow signal intensity on aT1-weighted image (A)

bull Bright signal intensity on aT2-weighted image (B)

bull Gadolinium-enhancedbrain MR images showheterogeneously strongenhancement (C and D)

bull Leptomeningealdisseminations at thecervical spine levels

10202016 22unusual giomas

bull NO standard careprotocols have beendefined

bull As these are newlydefined and raretumors

bull Surgical resection isfavoured if locationfavours

bull Mean progression freesurvival is 25 months

bull Mean overall survival is60 months

bull Reduced survival isbecause of inability toachieve gross resection

10202016 23unusual giomas

bull The usual indications foradjuvant treatment(chemotherapy orradiation therapy)include tumor recurrenceafter initial completeresection or symptomatictumors

bull In addition adjuvanttreatment is generallyindicated for tumors withgrowth on follow-upimaging even in theabsence of symptoms

bull The role of chemotherapyis in evolution

bull Chemotherapy may beimplemented for the treatment ofinoperable or partially resectedgliomas

bull The decision to use postoperativeadjuvant therapy also depends ona lesions perceived malignanttendencies as the potentialbenefits of radiation therapy andchemotherapy must be weighedagainst their morbidity

bull The decision to use postoperativeadjuvant therapy also depends ona lesions perceived malignanttendencies as the potentialbenefits of radiation therapy andchemotherapy must be weighedagainst their morbidity

10202016 24unusual giomas

Pleomorphic xanthoastrocytoma

bull First described by Kepsand colleuges in 1979

bull Represents lt1 of allastrocytomas

bull May be found alongentire neuraxis mostlysupratentorial( temporallobe)

bull Cerebellum and soinalcord are the other sites

bull Pts present in 2nd and 3rd

decadesbull Median age at 14 yearsbull Both sexes are affected

equallybull 70-80 pts present with

seizures and headachefocal neuro deficits

bull Rarely tumor mayhemmorrhage

10202016 25unusual giomas

bull Supratentorail are mostoften cortical and meningesbased

bull Chronic seizuresbull Grossely these are firm

tumors variable in colorrelatively avascular

bull Typically invade the piaarachnoid space

bull 13 pts have involvementof all the three meninges

bull Cyst fluid is xanthochromic

bull Very specific microscpoiccriteria for diagnosis are

1 Pleomorphic andxanthomatous astrocytes

2 Perivascular lymphocytes3 Non infilterating histology4 Reticulin stainingbull Pleomorphism can be as

vast as glioblastoma orsarcomatous tumors

10202016 26unusual giomas

bull IHC Staining revealsspindle cells and roundpleomorphic cellspositive for S-100 andGFAP

bull Staining forsynaptophysin and NSEmay be also present

bull Some workers havereported them to be asubtype of ganglioglioma

bull Association between theNeurofibromatosis Sturge Weber Syndrome

bull Loss of chr 9 and gain ofchromosome 7 have beenfound

10202016 27unusual giomas

Imaging

bull CTbull PXAs are typically hypo

or isodense and may bewell or poorlydemarcated usually withno or little surroundingoedema

bull Calcification is rarebull Due to its superficial

location it may causescalloping of theoverlying bone

10202016 28unusual giomas

bull MRIbull T1

ndash solid component iso tohypointense cf grey matter

ndash cystic component low signalndash leptomeningeal involvement

seen in over 70 of casesbull T1 C+ (Gd)

ndash solid component usuallyenhances vividly

bull T2ndash solid component iso to

hyperintense cf grey matterndash cystic component high signalndash on T2 FLAIR sequence cystic

areas show hyperintensityrelative to CSF due to higherprotein contents

ndash little surrounding vasogenicoedema

10202016 29unusual giomas

Management amp Outcome

bull Quite rare tumorsbull Surgical excision appears to

be best favoured modalitybull Actuarial survival trates are

91 at 5 years and 82 at10 years

bull Median survival 18 years

bull Total resection should beattempted in every case asfar as possible

bull Resection of cyst lining isnot indicated

bull Data regarding the use ofadjuvant therapy at thistime are limited to few casereports only

bull Pts who had incompleteresection high mitoticindex necrosis should bereserved for strict vigilanceand CEMRI on follow ups

10202016 30unusual giomas

bull At progression repeatresection be attempted

bull Proression should betaken as aggressivetumor type

bull Malignanttransformation maynecessitate adjuvanttherapy

10202016 31unusual giomas

bull Br J Neurosurg 2013 Dec27(6)759-64 doi 103109026886972013776666 Epub 2013 Mar 20

bull Pleomorphic xanthoastrocytoma long-term results of surgical treatment and analysis of prognosticfactors

bull Gallo P1 Cecchi PC Locatelli F Rizzo P Ghimenton C Gerosa M Pinna G

bull BACKGROUND

bull Pleomorphic Xanthoastrocytoma (PXA) is a rare brain tumour most commonly affecting children andyoung adults To date only few data regarding the long-term follow-up of these patients after surgery are

available The aim of this study is to describe our single-institution experience in the surgicalmanagement of this particular glioma over a period of over 18 years

bull METHODS

bull We performed a retrospective review of all cases of PXA (40 patients) operated upon at theDepartment of Neurosurgery of Verona Italy between 1990 and 2008 The impact of clinical radiologicalsurgical and histological factors on overall survival (OS) and progression-free survival (PFS) was analysed bymeans of univariate and multivariate models

bull FINDINGS

bull We achieved a gross total resection (GTR) in 65 of patients Histological diagnosis was of grade II in 80anaplastic features were present in the remaining 20 Adjuvant treatment radiotherapy or chemo-

radiotherapy was administered in 40 of the cases Median follow-up was 74 months OS at 5- and10 years was 7632 and 6824 respectively PFS at 5- and 10 years was 71 and 58respectively In the multivariate model histological grade extent of resection and age at diagnosis (le 30years vs gt 30 years) were the only independent prognostic factors for both OS and PFS

bull CONCLUSIONS

bull Our retrospective long-term study confirms the relatively favourable prognosis associatedwith PXA Young patients with a low-grade tumour (WHO grade II) who underwent GTR carrythe longest OS and PFS

10202016 32unusual giomas

bull Brain Tumor Pathol 2006 Apr23(1)55-63

bull A case of anaplastic pleomorphic xanthoastrocytoma presenting with tumor bleeding and cerebrospinal fluid dissemination

bull Asano K1 Miyamoto S Kubo O Kikkukawa T Yagihashi A Ohkuma H

bull Pleomorphic xanthoastrocytoma (PXA) has been considered an astrocytic tumorwith a relatively favorable prognosis However PXA cases having several recurrent patterns with poor prognosis have been reported in recent years and a new concept of anaplastic PXA has been proposed The present case was a 59-year-old woman who presented with tumor bleeding onset and cerebrospinal fluid dissemination The patient had sudden-onset right hemiparesis aphasia and consciousness disturbance and was admitted to a local area hospital After emergency surgery had removed the hematoma postoperative contrast-enhanced CT scan revealed a left temporal tumor A second surgery was therefore performed for initial tumor removal 2 months later Histopathological findings showed that the tumor was typical PXA with strong pleomorphism and xanthomatous changes and contained an ependymoma-like component in the center area However endothelial proliferation and mitosis were more remarkable compared to ordinary PXA The MIB-1 labeling index was 98 high From these findings the histopathological diagnosis was anaplastic PXA The patient underwent surgery to remove recurrent tumors 5 and 16 months later The patient died 36 months after the first onset and CT revealed glioblastoma-like

findings and cerebrospinal fluid dissemination This case report is the first case in which PXA presented with tumor bleeding onset Histopathological findings suggested anaplastic PXA from the first surgical specimens and PXA recurred many times We thus believe that the patient displayed primary anaplastic PXA rather than secondary anaplastic PXA that results in malignant transformation10202016 33unusual giomas

Ganglioglioma

bull WHO grade 1bull Initially described by

Perkins in 1926bull Benign well

differentiatedneuroepithelial tumors

bull Most commonly found intemporal lobe though canoccur anywhere in CNS

bull Commonly found inyoung patients withepilepsy

bull Most commonly notedtumor in temporal lobeepilepsy patients

bull Have been described inbrain steem spinal cordand cerebellum

bull Account for 1 ofprimary brain tumors

10202016 34unusual giomas

bull Male dominencebull Most common features

are seizures headachedizziness ataxiaprogressive weakness

bull Malignanttransformation is rare

bull Histology showsimmature mixture ofabnormal neural andglial elements

bull Macroscopically appearsolid or cystic

bull Cystic are welldelineatedfromsurrounding brain

bull Calcification is common

10202016 35unusual giomas

bull IHC staing shows reactivity for GFAP S-100 andvimentin

bull Neurons show synaptophysin class III betatubulin NFP chromogranin reactivity

10202016 36unusual giomas

Imaging

bull Highly variable imagingcharacters

bull No special typicalimaging criteria

bull Enhancement patternsare highly variable

bull Iso to hypointense onT1WI

bull Heterogenous andhyperintense on T2WI

bull Can contain solid cyticcalcified components

10202016 37unusual giomas

bull FDG PET scan showshypometabolism oreumetabolism in thesepatients

10202016 38unusual giomas

Management And Outcome

bull Gold standard is grosstotal resection

bull Seizure control aftersurgery is as high as80

bull Survival rates are highbull Event free survival of

low grade and highgrade gangiogioma are57 vs 15

bull The optimal surgicalresection depends uponthe eloquent areasinvolved in the vicinityof the tumor

bull The use ofchemotherapy is notwell established

bull Typically they shouldbe allowed for chemoand radiotherapy

10202016 39unusual giomas

bull The use of adjuvant therapy is warranted in thosecases which harbour residual lesions or features ofanaplasia

bull Potential harm to nearby brain areas preclude thejudicious use of radiation

10202016 40unusual giomas

bull Brain Tumor Res Treat 2014 Oct 2(2) 49ndash55

bull Treatment and Outcomes for Gangliogliomas A Single-Center Review of 16 Patients

bull Jye Young Song Jeong Hoon Kim Young Hyun Cho Chang Jin Kim and Eun Jung Lee

bull Ganglioglioma is a rare and slowly growing benign tumor We investigated the outcomes of patients who underwent different combination treatments

bull Methods

bull Between 1998 and 2012 16 patients including 11 men and 5 women with a median age of 125 years (range 25-65 years) were treated for intracranial gangliogliomas at our institution The median follow-up period was 57 years (range 48 days-156 years) Fifteen cases were included in the outcome assessment because one patient was lost to follow-up Complete resection was achieved in 8 (53) patients Six (40) patients underwent incomplete resection with or without adjuvant radiotherapy and one patient with a brainstem tumor underwent only stereotactic biopsy

bull Results

bull Gangliogliomas predominantly affected young (875) male patients and most frequently presented with seizures (64) Of eight patients who underwent complete resection seven did not show recurrence whereas only three of six with incomplete resection showed no recurrence Four patients with recurrence received salvage treatments (two repeat surgeries and two radiosurgeries) A tumor control rate of 93 (1415) was achieved at the last follow-up No recurrence or malignant changes were observed after a median follow-up of 12 and 45 years in four patients who received gamma knife (GK) radiosurgery as adjuvant and salvage treatment

bull Conclusion

bull Complete resection produced the best outcomes and incomplete resection followed by adjuvant or salvage treatments showed favorable outcomes In patients who are not eligible for complete resection because of tumor location or potential neurologic deficits following surgery GK radiosurgery should be considered for the treatment of residual or recurrent tumors

10202016 41unusual giomas

Papillary tumours of pineal

bull WHO grade II AND IIIbull Relatively new edition to WHObull They are equal in both sexesbull Reach medical attention due to headache mental

status changes and altered visionbull Contain eosinophilic cells with distinctive

borders with large pleomorphic nucleibull Positive for cytokeratin synaptophysin stain

variably for GFAP vimentin S-100

10202016 unusual giomas 42

Imaging

bull Generally found to be diffusely enhancinglesion of pineal region

bull Post 3rd ventricle with HCPbull Appear well demarcated from thalamus and

cerebellum

10202016 unusual giomas 43

Mangement

bull Standard treatment is excisionbull Grow slowly and donot transform to

malignant tumorsbull Local recurrence is however possiblebull Radiotherapy appears to provide good control

in local recurrence

10202016 unusual giomas 44

Choroid Glioma Of Third Ventricle

bull WHO Grade IIbull Ist recognised by WHO in

1998bull Arise in the anterior third

ventriclebull Commonly come to

attention after HCPbull Appera to contain in 3rd

ventricle and thought toarise from ventricularsurface

bull Composed of GFAP andvimentin positive cells inmucinous matrix

bull Nuclei are uniform insize and shape

bull Indolent growthbull May show EGF receptor

positivitybull Some suggets these tumor

arise from the lminaterminalis andventricular tanycytes

10202016 45unusual giomas

Imaging

bull Commonly fill 3rd

ventriclebull Diffusely enhance with

contrastbull Appear to infiltrate the

thalamusbull Large tumors may be

difficult to differentiatefrom suprasellar masses

10202016 46unusual giomas

10202016 47unusual giomas

bull Surgical management is limited by locationbull Access through the dialted ventricle and lamina

terminalis may be challangingbull Subtotal resection is thus commonbull Role of adjuvant radiotherapy is unknown

10202016 48unusual giomas

bull Acta Neurochir (Wien) 2008 Nov150(11)1183-91 doi 101007s00701-008-0014-6 Epub

2008 Oct 21

bull Chordoid glioma of the third ventricle

bull Vanhauwaert DJ1 Clement F Van Dorpe J Deruytter MJKGROUND

bull Chordoid glioma is a rare tumour (World Health Organisation grade II) originating from the third ventricle

with both glial and chordoid features It was first described by Brat in 1998 Since there is no detailed

information available on the outcome after surgery and adjuvant treatment we reviewed the literature

bull METHODS

bull A literature search through PUBMED revealed 50 cases of chordoid glioma Most reports

were found in pathology journals Information on the postoperative course was sometimes

very limited We reviewed the available literature and studied in detail the presenting

symptoms mortality and postoperative complications in relation to the extent of resective

surgery as well as the importance of adjuvant treatment

bull CONCLUSIONS

bull Mortality in the immediate postoperative period is 32 and is higher after gross total

resection as compared to subtotal resection Non-fatal postoperative complications are

hypothalamic disorders and mental alterations Gross total resection is the treatment of

choice since no recurrence has been reported after macroscopically complete resection

but this is often difficult because of the location and adherence to the hypothalamus The

role of postoperative radiotherapy is uncertain There is some indication that radiosurgery

with or without conventional irradiation is superior to conventional radiation alone Planned

subtotal resection followed by stereotactic radiosurgery can be a safe and effective

alternative in a patient in whom gross total resection is considered to be too risky

There is no report on the use of chemotherapy in the treatment of chordoid gliomas More

information about the optimal treatment strategy is needed and more reports are also needed10202016 49unusual giomas

bull Neurologist 2011 Jan17(1)52bull Chordoid glioma a case report and literature reviewbull Liu WP1 Cheng JX Yi XC Zhen HN Fei Z Li Q Zhang Xbull chordoid glioma is a rare tumor (World Health Organization grade II) with both

glial and chordoid features often located in the suprasellar region and anterior third ventricle It was first described by Brat in 1998 Because there is no detailed information available from the clinical perspective we reviewed the literature

bull METHODS

bull a literature search through PUBMED and CNKI revealed 64 cases of chordoid glioma Information on the clinical course was very limited We reviewed the literature and studied the pathologic and imaging features postoperative mortality and morbidity in relation to surgical extension and approaches and the importance of adjuvant treatment

bull CONCLUSIONS bull mortality in the immediate postoperative period is 28 and postoperative

morbidity is 60 which are statistically higher after gross total resection as compared with subtotal resection Translamina terminalis approach is considered to be the best approach The current study cannot document that patients have longer survival and higher quality of life after gross total resection than subtotal resection The role of postoperative radiotherapy is uncertain and there is no report on the use of chemotherapy More information about the optimal treatment strategy is needed

10202016 50unusual giomas

Conclusion

bull They are identified by their unique morphologicalfeatures

bull Many of these have indolent course and identified byepilepsy

bull Potentail diagnosis should be considered in youngpatients with seizures and cortically based lesions

bull Correct diagnosis requires experiencedneuropathologisrt

bull As more experienced is gained more knowledgetowards better understanding shall provide betteroutcomes in these patients

10202016 unusual giomas 51

10202016 52unusual giomas

Content supportndash youmann neurosurgery textbook

Imaging

bull On CT the appearnacesvaries from poorlydefined hypodensetumor to welldemarcated and highlyenhancing masses

10202016 17unusual giomas

bull On MR image tumorsare hypointense towhite matter on T1

bull Hyperintense on T2bull Hyperintense on FLAIRbull Enhancement is seen

around the cystic centre

10202016 18unusual giomas

Management And Prognosis

bull Surgical resection is main treatmentbull Gross total resection can be easily achieved as

lesions are mostly in the cortical areasbull Therapeutic value is of radiation and

chemotherapy is uncertain as these are raretumors

bull Prediction of outcome is difficult as these remainindolent for a long time and may rapidlytransform to glioblastoma resulting in fataloutcomes

10202016 19unusual giomas

Pilomyxoid astrocytoma

bull Recently described tumorsbull Similarities with pilocytic

astrocytomasbull Until recently these were

grouped with PAbull These involve entire

neuraxisbull Mean age at preentation is

18 monthsbull Most are found in

hypothalamic ndash chiasmicregion

bull Pts present with features ofraised ICP

bull RAISED HEADCIRCUMFERENCE may beonly subtle sign in infants

bull Histologically consists ofmyxoid matrix with cells inloose fibrillary and myxoidbackground

bull Tumor cells formperivascular rosettes

10202016 20unusual giomas

bull Preferred sites includebull (1) optic nerve (lsquooptic

nerve gliomarsquo)bull (2) optic

chiasmhypothalamusbull (3) thalamus and basal

gangliabull (4) cerebral hemispherebull (5) cerebellum (lsquocerebellar

astrocytomarsquo) andbull (6) brainstem (dorsal

exophytic brainstemglioma)

bull Genetically chracterized bythe presence of NF1mutations BRAFduplications and theabsence of IDH1 mutation

10202016 21unusual giomas

Imaging

bull The huge chiasmatic-hypothalamic tumor revealslow signal intensity on aT1-weighted image (A)

bull Bright signal intensity on aT2-weighted image (B)

bull Gadolinium-enhancedbrain MR images showheterogeneously strongenhancement (C and D)

bull Leptomeningealdisseminations at thecervical spine levels

10202016 22unusual giomas

bull NO standard careprotocols have beendefined

bull As these are newlydefined and raretumors

bull Surgical resection isfavoured if locationfavours

bull Mean progression freesurvival is 25 months

bull Mean overall survival is60 months

bull Reduced survival isbecause of inability toachieve gross resection

10202016 23unusual giomas

bull The usual indications foradjuvant treatment(chemotherapy orradiation therapy)include tumor recurrenceafter initial completeresection or symptomatictumors

bull In addition adjuvanttreatment is generallyindicated for tumors withgrowth on follow-upimaging even in theabsence of symptoms

bull The role of chemotherapyis in evolution

bull Chemotherapy may beimplemented for the treatment ofinoperable or partially resectedgliomas

bull The decision to use postoperativeadjuvant therapy also depends ona lesions perceived malignanttendencies as the potentialbenefits of radiation therapy andchemotherapy must be weighedagainst their morbidity

bull The decision to use postoperativeadjuvant therapy also depends ona lesions perceived malignanttendencies as the potentialbenefits of radiation therapy andchemotherapy must be weighedagainst their morbidity

10202016 24unusual giomas

Pleomorphic xanthoastrocytoma

bull First described by Kepsand colleuges in 1979

bull Represents lt1 of allastrocytomas

bull May be found alongentire neuraxis mostlysupratentorial( temporallobe)

bull Cerebellum and soinalcord are the other sites

bull Pts present in 2nd and 3rd

decadesbull Median age at 14 yearsbull Both sexes are affected

equallybull 70-80 pts present with

seizures and headachefocal neuro deficits

bull Rarely tumor mayhemmorrhage

10202016 25unusual giomas

bull Supratentorail are mostoften cortical and meningesbased

bull Chronic seizuresbull Grossely these are firm

tumors variable in colorrelatively avascular

bull Typically invade the piaarachnoid space

bull 13 pts have involvementof all the three meninges

bull Cyst fluid is xanthochromic

bull Very specific microscpoiccriteria for diagnosis are

1 Pleomorphic andxanthomatous astrocytes

2 Perivascular lymphocytes3 Non infilterating histology4 Reticulin stainingbull Pleomorphism can be as

vast as glioblastoma orsarcomatous tumors

10202016 26unusual giomas

bull IHC Staining revealsspindle cells and roundpleomorphic cellspositive for S-100 andGFAP

bull Staining forsynaptophysin and NSEmay be also present

bull Some workers havereported them to be asubtype of ganglioglioma

bull Association between theNeurofibromatosis Sturge Weber Syndrome

bull Loss of chr 9 and gain ofchromosome 7 have beenfound

10202016 27unusual giomas

Imaging

bull CTbull PXAs are typically hypo

or isodense and may bewell or poorlydemarcated usually withno or little surroundingoedema

bull Calcification is rarebull Due to its superficial

location it may causescalloping of theoverlying bone

10202016 28unusual giomas

bull MRIbull T1

ndash solid component iso tohypointense cf grey matter

ndash cystic component low signalndash leptomeningeal involvement

seen in over 70 of casesbull T1 C+ (Gd)

ndash solid component usuallyenhances vividly

bull T2ndash solid component iso to

hyperintense cf grey matterndash cystic component high signalndash on T2 FLAIR sequence cystic

areas show hyperintensityrelative to CSF due to higherprotein contents

ndash little surrounding vasogenicoedema

10202016 29unusual giomas

Management amp Outcome

bull Quite rare tumorsbull Surgical excision appears to

be best favoured modalitybull Actuarial survival trates are

91 at 5 years and 82 at10 years

bull Median survival 18 years

bull Total resection should beattempted in every case asfar as possible

bull Resection of cyst lining isnot indicated

bull Data regarding the use ofadjuvant therapy at thistime are limited to few casereports only

bull Pts who had incompleteresection high mitoticindex necrosis should bereserved for strict vigilanceand CEMRI on follow ups

10202016 30unusual giomas

bull At progression repeatresection be attempted

bull Proression should betaken as aggressivetumor type

bull Malignanttransformation maynecessitate adjuvanttherapy

10202016 31unusual giomas

bull Br J Neurosurg 2013 Dec27(6)759-64 doi 103109026886972013776666 Epub 2013 Mar 20

bull Pleomorphic xanthoastrocytoma long-term results of surgical treatment and analysis of prognosticfactors

bull Gallo P1 Cecchi PC Locatelli F Rizzo P Ghimenton C Gerosa M Pinna G

bull BACKGROUND

bull Pleomorphic Xanthoastrocytoma (PXA) is a rare brain tumour most commonly affecting children andyoung adults To date only few data regarding the long-term follow-up of these patients after surgery are

available The aim of this study is to describe our single-institution experience in the surgicalmanagement of this particular glioma over a period of over 18 years

bull METHODS

bull We performed a retrospective review of all cases of PXA (40 patients) operated upon at theDepartment of Neurosurgery of Verona Italy between 1990 and 2008 The impact of clinical radiologicalsurgical and histological factors on overall survival (OS) and progression-free survival (PFS) was analysed bymeans of univariate and multivariate models

bull FINDINGS

bull We achieved a gross total resection (GTR) in 65 of patients Histological diagnosis was of grade II in 80anaplastic features were present in the remaining 20 Adjuvant treatment radiotherapy or chemo-

radiotherapy was administered in 40 of the cases Median follow-up was 74 months OS at 5- and10 years was 7632 and 6824 respectively PFS at 5- and 10 years was 71 and 58respectively In the multivariate model histological grade extent of resection and age at diagnosis (le 30years vs gt 30 years) were the only independent prognostic factors for both OS and PFS

bull CONCLUSIONS

bull Our retrospective long-term study confirms the relatively favourable prognosis associatedwith PXA Young patients with a low-grade tumour (WHO grade II) who underwent GTR carrythe longest OS and PFS

10202016 32unusual giomas

bull Brain Tumor Pathol 2006 Apr23(1)55-63

bull A case of anaplastic pleomorphic xanthoastrocytoma presenting with tumor bleeding and cerebrospinal fluid dissemination

bull Asano K1 Miyamoto S Kubo O Kikkukawa T Yagihashi A Ohkuma H

bull Pleomorphic xanthoastrocytoma (PXA) has been considered an astrocytic tumorwith a relatively favorable prognosis However PXA cases having several recurrent patterns with poor prognosis have been reported in recent years and a new concept of anaplastic PXA has been proposed The present case was a 59-year-old woman who presented with tumor bleeding onset and cerebrospinal fluid dissemination The patient had sudden-onset right hemiparesis aphasia and consciousness disturbance and was admitted to a local area hospital After emergency surgery had removed the hematoma postoperative contrast-enhanced CT scan revealed a left temporal tumor A second surgery was therefore performed for initial tumor removal 2 months later Histopathological findings showed that the tumor was typical PXA with strong pleomorphism and xanthomatous changes and contained an ependymoma-like component in the center area However endothelial proliferation and mitosis were more remarkable compared to ordinary PXA The MIB-1 labeling index was 98 high From these findings the histopathological diagnosis was anaplastic PXA The patient underwent surgery to remove recurrent tumors 5 and 16 months later The patient died 36 months after the first onset and CT revealed glioblastoma-like

findings and cerebrospinal fluid dissemination This case report is the first case in which PXA presented with tumor bleeding onset Histopathological findings suggested anaplastic PXA from the first surgical specimens and PXA recurred many times We thus believe that the patient displayed primary anaplastic PXA rather than secondary anaplastic PXA that results in malignant transformation10202016 33unusual giomas

Ganglioglioma

bull WHO grade 1bull Initially described by

Perkins in 1926bull Benign well

differentiatedneuroepithelial tumors

bull Most commonly found intemporal lobe though canoccur anywhere in CNS

bull Commonly found inyoung patients withepilepsy

bull Most commonly notedtumor in temporal lobeepilepsy patients

bull Have been described inbrain steem spinal cordand cerebellum

bull Account for 1 ofprimary brain tumors

10202016 34unusual giomas

bull Male dominencebull Most common features

are seizures headachedizziness ataxiaprogressive weakness

bull Malignanttransformation is rare

bull Histology showsimmature mixture ofabnormal neural andglial elements

bull Macroscopically appearsolid or cystic

bull Cystic are welldelineatedfromsurrounding brain

bull Calcification is common

10202016 35unusual giomas

bull IHC staing shows reactivity for GFAP S-100 andvimentin

bull Neurons show synaptophysin class III betatubulin NFP chromogranin reactivity

10202016 36unusual giomas

Imaging

bull Highly variable imagingcharacters

bull No special typicalimaging criteria

bull Enhancement patternsare highly variable

bull Iso to hypointense onT1WI

bull Heterogenous andhyperintense on T2WI

bull Can contain solid cyticcalcified components

10202016 37unusual giomas

bull FDG PET scan showshypometabolism oreumetabolism in thesepatients

10202016 38unusual giomas

Management And Outcome

bull Gold standard is grosstotal resection

bull Seizure control aftersurgery is as high as80

bull Survival rates are highbull Event free survival of

low grade and highgrade gangiogioma are57 vs 15

bull The optimal surgicalresection depends uponthe eloquent areasinvolved in the vicinityof the tumor

bull The use ofchemotherapy is notwell established

bull Typically they shouldbe allowed for chemoand radiotherapy

10202016 39unusual giomas

bull The use of adjuvant therapy is warranted in thosecases which harbour residual lesions or features ofanaplasia

bull Potential harm to nearby brain areas preclude thejudicious use of radiation

10202016 40unusual giomas

bull Brain Tumor Res Treat 2014 Oct 2(2) 49ndash55

bull Treatment and Outcomes for Gangliogliomas A Single-Center Review of 16 Patients

bull Jye Young Song Jeong Hoon Kim Young Hyun Cho Chang Jin Kim and Eun Jung Lee

bull Ganglioglioma is a rare and slowly growing benign tumor We investigated the outcomes of patients who underwent different combination treatments

bull Methods

bull Between 1998 and 2012 16 patients including 11 men and 5 women with a median age of 125 years (range 25-65 years) were treated for intracranial gangliogliomas at our institution The median follow-up period was 57 years (range 48 days-156 years) Fifteen cases were included in the outcome assessment because one patient was lost to follow-up Complete resection was achieved in 8 (53) patients Six (40) patients underwent incomplete resection with or without adjuvant radiotherapy and one patient with a brainstem tumor underwent only stereotactic biopsy

bull Results

bull Gangliogliomas predominantly affected young (875) male patients and most frequently presented with seizures (64) Of eight patients who underwent complete resection seven did not show recurrence whereas only three of six with incomplete resection showed no recurrence Four patients with recurrence received salvage treatments (two repeat surgeries and two radiosurgeries) A tumor control rate of 93 (1415) was achieved at the last follow-up No recurrence or malignant changes were observed after a median follow-up of 12 and 45 years in four patients who received gamma knife (GK) radiosurgery as adjuvant and salvage treatment

bull Conclusion

bull Complete resection produced the best outcomes and incomplete resection followed by adjuvant or salvage treatments showed favorable outcomes In patients who are not eligible for complete resection because of tumor location or potential neurologic deficits following surgery GK radiosurgery should be considered for the treatment of residual or recurrent tumors

10202016 41unusual giomas

Papillary tumours of pineal

bull WHO grade II AND IIIbull Relatively new edition to WHObull They are equal in both sexesbull Reach medical attention due to headache mental

status changes and altered visionbull Contain eosinophilic cells with distinctive

borders with large pleomorphic nucleibull Positive for cytokeratin synaptophysin stain

variably for GFAP vimentin S-100

10202016 unusual giomas 42

Imaging

bull Generally found to be diffusely enhancinglesion of pineal region

bull Post 3rd ventricle with HCPbull Appear well demarcated from thalamus and

cerebellum

10202016 unusual giomas 43

Mangement

bull Standard treatment is excisionbull Grow slowly and donot transform to

malignant tumorsbull Local recurrence is however possiblebull Radiotherapy appears to provide good control

in local recurrence

10202016 unusual giomas 44

Choroid Glioma Of Third Ventricle

bull WHO Grade IIbull Ist recognised by WHO in

1998bull Arise in the anterior third

ventriclebull Commonly come to

attention after HCPbull Appera to contain in 3rd

ventricle and thought toarise from ventricularsurface

bull Composed of GFAP andvimentin positive cells inmucinous matrix

bull Nuclei are uniform insize and shape

bull Indolent growthbull May show EGF receptor

positivitybull Some suggets these tumor

arise from the lminaterminalis andventricular tanycytes

10202016 45unusual giomas

Imaging

bull Commonly fill 3rd

ventriclebull Diffusely enhance with

contrastbull Appear to infiltrate the

thalamusbull Large tumors may be

difficult to differentiatefrom suprasellar masses

10202016 46unusual giomas

10202016 47unusual giomas

bull Surgical management is limited by locationbull Access through the dialted ventricle and lamina

terminalis may be challangingbull Subtotal resection is thus commonbull Role of adjuvant radiotherapy is unknown

10202016 48unusual giomas

bull Acta Neurochir (Wien) 2008 Nov150(11)1183-91 doi 101007s00701-008-0014-6 Epub

2008 Oct 21

bull Chordoid glioma of the third ventricle

bull Vanhauwaert DJ1 Clement F Van Dorpe J Deruytter MJKGROUND

bull Chordoid glioma is a rare tumour (World Health Organisation grade II) originating from the third ventricle

with both glial and chordoid features It was first described by Brat in 1998 Since there is no detailed

information available on the outcome after surgery and adjuvant treatment we reviewed the literature

bull METHODS

bull A literature search through PUBMED revealed 50 cases of chordoid glioma Most reports

were found in pathology journals Information on the postoperative course was sometimes

very limited We reviewed the available literature and studied in detail the presenting

symptoms mortality and postoperative complications in relation to the extent of resective

surgery as well as the importance of adjuvant treatment

bull CONCLUSIONS

bull Mortality in the immediate postoperative period is 32 and is higher after gross total

resection as compared to subtotal resection Non-fatal postoperative complications are

hypothalamic disorders and mental alterations Gross total resection is the treatment of

choice since no recurrence has been reported after macroscopically complete resection

but this is often difficult because of the location and adherence to the hypothalamus The

role of postoperative radiotherapy is uncertain There is some indication that radiosurgery

with or without conventional irradiation is superior to conventional radiation alone Planned

subtotal resection followed by stereotactic radiosurgery can be a safe and effective

alternative in a patient in whom gross total resection is considered to be too risky

There is no report on the use of chemotherapy in the treatment of chordoid gliomas More

information about the optimal treatment strategy is needed and more reports are also needed10202016 49unusual giomas

bull Neurologist 2011 Jan17(1)52bull Chordoid glioma a case report and literature reviewbull Liu WP1 Cheng JX Yi XC Zhen HN Fei Z Li Q Zhang Xbull chordoid glioma is a rare tumor (World Health Organization grade II) with both

glial and chordoid features often located in the suprasellar region and anterior third ventricle It was first described by Brat in 1998 Because there is no detailed information available from the clinical perspective we reviewed the literature

bull METHODS

bull a literature search through PUBMED and CNKI revealed 64 cases of chordoid glioma Information on the clinical course was very limited We reviewed the literature and studied the pathologic and imaging features postoperative mortality and morbidity in relation to surgical extension and approaches and the importance of adjuvant treatment

bull CONCLUSIONS bull mortality in the immediate postoperative period is 28 and postoperative

morbidity is 60 which are statistically higher after gross total resection as compared with subtotal resection Translamina terminalis approach is considered to be the best approach The current study cannot document that patients have longer survival and higher quality of life after gross total resection than subtotal resection The role of postoperative radiotherapy is uncertain and there is no report on the use of chemotherapy More information about the optimal treatment strategy is needed

10202016 50unusual giomas

Conclusion

bull They are identified by their unique morphologicalfeatures

bull Many of these have indolent course and identified byepilepsy

bull Potentail diagnosis should be considered in youngpatients with seizures and cortically based lesions

bull Correct diagnosis requires experiencedneuropathologisrt

bull As more experienced is gained more knowledgetowards better understanding shall provide betteroutcomes in these patients

10202016 unusual giomas 51

10202016 52unusual giomas

Content supportndash youmann neurosurgery textbook

bull On MR image tumorsare hypointense towhite matter on T1

bull Hyperintense on T2bull Hyperintense on FLAIRbull Enhancement is seen

around the cystic centre

10202016 18unusual giomas

Management And Prognosis

bull Surgical resection is main treatmentbull Gross total resection can be easily achieved as

lesions are mostly in the cortical areasbull Therapeutic value is of radiation and

chemotherapy is uncertain as these are raretumors

bull Prediction of outcome is difficult as these remainindolent for a long time and may rapidlytransform to glioblastoma resulting in fataloutcomes

10202016 19unusual giomas

Pilomyxoid astrocytoma

bull Recently described tumorsbull Similarities with pilocytic

astrocytomasbull Until recently these were

grouped with PAbull These involve entire

neuraxisbull Mean age at preentation is

18 monthsbull Most are found in

hypothalamic ndash chiasmicregion

bull Pts present with features ofraised ICP

bull RAISED HEADCIRCUMFERENCE may beonly subtle sign in infants

bull Histologically consists ofmyxoid matrix with cells inloose fibrillary and myxoidbackground

bull Tumor cells formperivascular rosettes

10202016 20unusual giomas

bull Preferred sites includebull (1) optic nerve (lsquooptic

nerve gliomarsquo)bull (2) optic

chiasmhypothalamusbull (3) thalamus and basal

gangliabull (4) cerebral hemispherebull (5) cerebellum (lsquocerebellar

astrocytomarsquo) andbull (6) brainstem (dorsal

exophytic brainstemglioma)

bull Genetically chracterized bythe presence of NF1mutations BRAFduplications and theabsence of IDH1 mutation

10202016 21unusual giomas

Imaging

bull The huge chiasmatic-hypothalamic tumor revealslow signal intensity on aT1-weighted image (A)

bull Bright signal intensity on aT2-weighted image (B)

bull Gadolinium-enhancedbrain MR images showheterogeneously strongenhancement (C and D)

bull Leptomeningealdisseminations at thecervical spine levels

10202016 22unusual giomas

bull NO standard careprotocols have beendefined

bull As these are newlydefined and raretumors

bull Surgical resection isfavoured if locationfavours

bull Mean progression freesurvival is 25 months

bull Mean overall survival is60 months

bull Reduced survival isbecause of inability toachieve gross resection

10202016 23unusual giomas

bull The usual indications foradjuvant treatment(chemotherapy orradiation therapy)include tumor recurrenceafter initial completeresection or symptomatictumors

bull In addition adjuvanttreatment is generallyindicated for tumors withgrowth on follow-upimaging even in theabsence of symptoms

bull The role of chemotherapyis in evolution

bull Chemotherapy may beimplemented for the treatment ofinoperable or partially resectedgliomas

bull The decision to use postoperativeadjuvant therapy also depends ona lesions perceived malignanttendencies as the potentialbenefits of radiation therapy andchemotherapy must be weighedagainst their morbidity

bull The decision to use postoperativeadjuvant therapy also depends ona lesions perceived malignanttendencies as the potentialbenefits of radiation therapy andchemotherapy must be weighedagainst their morbidity

10202016 24unusual giomas

Pleomorphic xanthoastrocytoma

bull First described by Kepsand colleuges in 1979

bull Represents lt1 of allastrocytomas

bull May be found alongentire neuraxis mostlysupratentorial( temporallobe)

bull Cerebellum and soinalcord are the other sites

bull Pts present in 2nd and 3rd

decadesbull Median age at 14 yearsbull Both sexes are affected

equallybull 70-80 pts present with

seizures and headachefocal neuro deficits

bull Rarely tumor mayhemmorrhage

10202016 25unusual giomas

bull Supratentorail are mostoften cortical and meningesbased

bull Chronic seizuresbull Grossely these are firm

tumors variable in colorrelatively avascular

bull Typically invade the piaarachnoid space

bull 13 pts have involvementof all the three meninges

bull Cyst fluid is xanthochromic

bull Very specific microscpoiccriteria for diagnosis are

1 Pleomorphic andxanthomatous astrocytes

2 Perivascular lymphocytes3 Non infilterating histology4 Reticulin stainingbull Pleomorphism can be as

vast as glioblastoma orsarcomatous tumors

10202016 26unusual giomas

bull IHC Staining revealsspindle cells and roundpleomorphic cellspositive for S-100 andGFAP

bull Staining forsynaptophysin and NSEmay be also present

bull Some workers havereported them to be asubtype of ganglioglioma

bull Association between theNeurofibromatosis Sturge Weber Syndrome

bull Loss of chr 9 and gain ofchromosome 7 have beenfound

10202016 27unusual giomas

Imaging

bull CTbull PXAs are typically hypo

or isodense and may bewell or poorlydemarcated usually withno or little surroundingoedema

bull Calcification is rarebull Due to its superficial

location it may causescalloping of theoverlying bone

10202016 28unusual giomas

bull MRIbull T1

ndash solid component iso tohypointense cf grey matter

ndash cystic component low signalndash leptomeningeal involvement

seen in over 70 of casesbull T1 C+ (Gd)

ndash solid component usuallyenhances vividly

bull T2ndash solid component iso to

hyperintense cf grey matterndash cystic component high signalndash on T2 FLAIR sequence cystic

areas show hyperintensityrelative to CSF due to higherprotein contents

ndash little surrounding vasogenicoedema

10202016 29unusual giomas

Management amp Outcome

bull Quite rare tumorsbull Surgical excision appears to

be best favoured modalitybull Actuarial survival trates are

91 at 5 years and 82 at10 years

bull Median survival 18 years

bull Total resection should beattempted in every case asfar as possible

bull Resection of cyst lining isnot indicated

bull Data regarding the use ofadjuvant therapy at thistime are limited to few casereports only

bull Pts who had incompleteresection high mitoticindex necrosis should bereserved for strict vigilanceand CEMRI on follow ups

10202016 30unusual giomas

bull At progression repeatresection be attempted

bull Proression should betaken as aggressivetumor type

bull Malignanttransformation maynecessitate adjuvanttherapy

10202016 31unusual giomas

bull Br J Neurosurg 2013 Dec27(6)759-64 doi 103109026886972013776666 Epub 2013 Mar 20

bull Pleomorphic xanthoastrocytoma long-term results of surgical treatment and analysis of prognosticfactors

bull Gallo P1 Cecchi PC Locatelli F Rizzo P Ghimenton C Gerosa M Pinna G

bull BACKGROUND

bull Pleomorphic Xanthoastrocytoma (PXA) is a rare brain tumour most commonly affecting children andyoung adults To date only few data regarding the long-term follow-up of these patients after surgery are

available The aim of this study is to describe our single-institution experience in the surgicalmanagement of this particular glioma over a period of over 18 years

bull METHODS

bull We performed a retrospective review of all cases of PXA (40 patients) operated upon at theDepartment of Neurosurgery of Verona Italy between 1990 and 2008 The impact of clinical radiologicalsurgical and histological factors on overall survival (OS) and progression-free survival (PFS) was analysed bymeans of univariate and multivariate models

bull FINDINGS

bull We achieved a gross total resection (GTR) in 65 of patients Histological diagnosis was of grade II in 80anaplastic features were present in the remaining 20 Adjuvant treatment radiotherapy or chemo-

radiotherapy was administered in 40 of the cases Median follow-up was 74 months OS at 5- and10 years was 7632 and 6824 respectively PFS at 5- and 10 years was 71 and 58respectively In the multivariate model histological grade extent of resection and age at diagnosis (le 30years vs gt 30 years) were the only independent prognostic factors for both OS and PFS

bull CONCLUSIONS

bull Our retrospective long-term study confirms the relatively favourable prognosis associatedwith PXA Young patients with a low-grade tumour (WHO grade II) who underwent GTR carrythe longest OS and PFS

10202016 32unusual giomas

bull Brain Tumor Pathol 2006 Apr23(1)55-63

bull A case of anaplastic pleomorphic xanthoastrocytoma presenting with tumor bleeding and cerebrospinal fluid dissemination

bull Asano K1 Miyamoto S Kubo O Kikkukawa T Yagihashi A Ohkuma H

bull Pleomorphic xanthoastrocytoma (PXA) has been considered an astrocytic tumorwith a relatively favorable prognosis However PXA cases having several recurrent patterns with poor prognosis have been reported in recent years and a new concept of anaplastic PXA has been proposed The present case was a 59-year-old woman who presented with tumor bleeding onset and cerebrospinal fluid dissemination The patient had sudden-onset right hemiparesis aphasia and consciousness disturbance and was admitted to a local area hospital After emergency surgery had removed the hematoma postoperative contrast-enhanced CT scan revealed a left temporal tumor A second surgery was therefore performed for initial tumor removal 2 months later Histopathological findings showed that the tumor was typical PXA with strong pleomorphism and xanthomatous changes and contained an ependymoma-like component in the center area However endothelial proliferation and mitosis were more remarkable compared to ordinary PXA The MIB-1 labeling index was 98 high From these findings the histopathological diagnosis was anaplastic PXA The patient underwent surgery to remove recurrent tumors 5 and 16 months later The patient died 36 months after the first onset and CT revealed glioblastoma-like

findings and cerebrospinal fluid dissemination This case report is the first case in which PXA presented with tumor bleeding onset Histopathological findings suggested anaplastic PXA from the first surgical specimens and PXA recurred many times We thus believe that the patient displayed primary anaplastic PXA rather than secondary anaplastic PXA that results in malignant transformation10202016 33unusual giomas

Ganglioglioma

bull WHO grade 1bull Initially described by

Perkins in 1926bull Benign well

differentiatedneuroepithelial tumors

bull Most commonly found intemporal lobe though canoccur anywhere in CNS

bull Commonly found inyoung patients withepilepsy

bull Most commonly notedtumor in temporal lobeepilepsy patients

bull Have been described inbrain steem spinal cordand cerebellum

bull Account for 1 ofprimary brain tumors

10202016 34unusual giomas

bull Male dominencebull Most common features

are seizures headachedizziness ataxiaprogressive weakness

bull Malignanttransformation is rare

bull Histology showsimmature mixture ofabnormal neural andglial elements

bull Macroscopically appearsolid or cystic

bull Cystic are welldelineatedfromsurrounding brain

bull Calcification is common

10202016 35unusual giomas

bull IHC staing shows reactivity for GFAP S-100 andvimentin

bull Neurons show synaptophysin class III betatubulin NFP chromogranin reactivity

10202016 36unusual giomas

Imaging

bull Highly variable imagingcharacters

bull No special typicalimaging criteria

bull Enhancement patternsare highly variable

bull Iso to hypointense onT1WI

bull Heterogenous andhyperintense on T2WI

bull Can contain solid cyticcalcified components

10202016 37unusual giomas

bull FDG PET scan showshypometabolism oreumetabolism in thesepatients

10202016 38unusual giomas

Management And Outcome

bull Gold standard is grosstotal resection

bull Seizure control aftersurgery is as high as80

bull Survival rates are highbull Event free survival of

low grade and highgrade gangiogioma are57 vs 15

bull The optimal surgicalresection depends uponthe eloquent areasinvolved in the vicinityof the tumor

bull The use ofchemotherapy is notwell established

bull Typically they shouldbe allowed for chemoand radiotherapy

10202016 39unusual giomas

bull The use of adjuvant therapy is warranted in thosecases which harbour residual lesions or features ofanaplasia

bull Potential harm to nearby brain areas preclude thejudicious use of radiation

10202016 40unusual giomas

bull Brain Tumor Res Treat 2014 Oct 2(2) 49ndash55

bull Treatment and Outcomes for Gangliogliomas A Single-Center Review of 16 Patients

bull Jye Young Song Jeong Hoon Kim Young Hyun Cho Chang Jin Kim and Eun Jung Lee

bull Ganglioglioma is a rare and slowly growing benign tumor We investigated the outcomes of patients who underwent different combination treatments

bull Methods

bull Between 1998 and 2012 16 patients including 11 men and 5 women with a median age of 125 years (range 25-65 years) were treated for intracranial gangliogliomas at our institution The median follow-up period was 57 years (range 48 days-156 years) Fifteen cases were included in the outcome assessment because one patient was lost to follow-up Complete resection was achieved in 8 (53) patients Six (40) patients underwent incomplete resection with or without adjuvant radiotherapy and one patient with a brainstem tumor underwent only stereotactic biopsy

bull Results

bull Gangliogliomas predominantly affected young (875) male patients and most frequently presented with seizures (64) Of eight patients who underwent complete resection seven did not show recurrence whereas only three of six with incomplete resection showed no recurrence Four patients with recurrence received salvage treatments (two repeat surgeries and two radiosurgeries) A tumor control rate of 93 (1415) was achieved at the last follow-up No recurrence or malignant changes were observed after a median follow-up of 12 and 45 years in four patients who received gamma knife (GK) radiosurgery as adjuvant and salvage treatment

bull Conclusion

bull Complete resection produced the best outcomes and incomplete resection followed by adjuvant or salvage treatments showed favorable outcomes In patients who are not eligible for complete resection because of tumor location or potential neurologic deficits following surgery GK radiosurgery should be considered for the treatment of residual or recurrent tumors

10202016 41unusual giomas

Papillary tumours of pineal

bull WHO grade II AND IIIbull Relatively new edition to WHObull They are equal in both sexesbull Reach medical attention due to headache mental

status changes and altered visionbull Contain eosinophilic cells with distinctive

borders with large pleomorphic nucleibull Positive for cytokeratin synaptophysin stain

variably for GFAP vimentin S-100

10202016 unusual giomas 42

Imaging

bull Generally found to be diffusely enhancinglesion of pineal region

bull Post 3rd ventricle with HCPbull Appear well demarcated from thalamus and

cerebellum

10202016 unusual giomas 43

Mangement

bull Standard treatment is excisionbull Grow slowly and donot transform to

malignant tumorsbull Local recurrence is however possiblebull Radiotherapy appears to provide good control

in local recurrence

10202016 unusual giomas 44

Choroid Glioma Of Third Ventricle

bull WHO Grade IIbull Ist recognised by WHO in

1998bull Arise in the anterior third

ventriclebull Commonly come to

attention after HCPbull Appera to contain in 3rd

ventricle and thought toarise from ventricularsurface

bull Composed of GFAP andvimentin positive cells inmucinous matrix

bull Nuclei are uniform insize and shape

bull Indolent growthbull May show EGF receptor

positivitybull Some suggets these tumor

arise from the lminaterminalis andventricular tanycytes

10202016 45unusual giomas

Imaging

bull Commonly fill 3rd

ventriclebull Diffusely enhance with

contrastbull Appear to infiltrate the

thalamusbull Large tumors may be

difficult to differentiatefrom suprasellar masses

10202016 46unusual giomas

10202016 47unusual giomas

bull Surgical management is limited by locationbull Access through the dialted ventricle and lamina

terminalis may be challangingbull Subtotal resection is thus commonbull Role of adjuvant radiotherapy is unknown

10202016 48unusual giomas

bull Acta Neurochir (Wien) 2008 Nov150(11)1183-91 doi 101007s00701-008-0014-6 Epub

2008 Oct 21

bull Chordoid glioma of the third ventricle

bull Vanhauwaert DJ1 Clement F Van Dorpe J Deruytter MJKGROUND

bull Chordoid glioma is a rare tumour (World Health Organisation grade II) originating from the third ventricle

with both glial and chordoid features It was first described by Brat in 1998 Since there is no detailed

information available on the outcome after surgery and adjuvant treatment we reviewed the literature

bull METHODS

bull A literature search through PUBMED revealed 50 cases of chordoid glioma Most reports

were found in pathology journals Information on the postoperative course was sometimes

very limited We reviewed the available literature and studied in detail the presenting

symptoms mortality and postoperative complications in relation to the extent of resective

surgery as well as the importance of adjuvant treatment

bull CONCLUSIONS

bull Mortality in the immediate postoperative period is 32 and is higher after gross total

resection as compared to subtotal resection Non-fatal postoperative complications are

hypothalamic disorders and mental alterations Gross total resection is the treatment of

choice since no recurrence has been reported after macroscopically complete resection

but this is often difficult because of the location and adherence to the hypothalamus The

role of postoperative radiotherapy is uncertain There is some indication that radiosurgery

with or without conventional irradiation is superior to conventional radiation alone Planned

subtotal resection followed by stereotactic radiosurgery can be a safe and effective

alternative in a patient in whom gross total resection is considered to be too risky

There is no report on the use of chemotherapy in the treatment of chordoid gliomas More

information about the optimal treatment strategy is needed and more reports are also needed10202016 49unusual giomas

bull Neurologist 2011 Jan17(1)52bull Chordoid glioma a case report and literature reviewbull Liu WP1 Cheng JX Yi XC Zhen HN Fei Z Li Q Zhang Xbull chordoid glioma is a rare tumor (World Health Organization grade II) with both

glial and chordoid features often located in the suprasellar region and anterior third ventricle It was first described by Brat in 1998 Because there is no detailed information available from the clinical perspective we reviewed the literature

bull METHODS

bull a literature search through PUBMED and CNKI revealed 64 cases of chordoid glioma Information on the clinical course was very limited We reviewed the literature and studied the pathologic and imaging features postoperative mortality and morbidity in relation to surgical extension and approaches and the importance of adjuvant treatment

bull CONCLUSIONS bull mortality in the immediate postoperative period is 28 and postoperative

morbidity is 60 which are statistically higher after gross total resection as compared with subtotal resection Translamina terminalis approach is considered to be the best approach The current study cannot document that patients have longer survival and higher quality of life after gross total resection than subtotal resection The role of postoperative radiotherapy is uncertain and there is no report on the use of chemotherapy More information about the optimal treatment strategy is needed

10202016 50unusual giomas

Conclusion

bull They are identified by their unique morphologicalfeatures

bull Many of these have indolent course and identified byepilepsy

bull Potentail diagnosis should be considered in youngpatients with seizures and cortically based lesions

bull Correct diagnosis requires experiencedneuropathologisrt

bull As more experienced is gained more knowledgetowards better understanding shall provide betteroutcomes in these patients

10202016 unusual giomas 51

10202016 52unusual giomas

Content supportndash youmann neurosurgery textbook

Management And Prognosis

bull Surgical resection is main treatmentbull Gross total resection can be easily achieved as

lesions are mostly in the cortical areasbull Therapeutic value is of radiation and

chemotherapy is uncertain as these are raretumors

bull Prediction of outcome is difficult as these remainindolent for a long time and may rapidlytransform to glioblastoma resulting in fataloutcomes

10202016 19unusual giomas

Pilomyxoid astrocytoma

bull Recently described tumorsbull Similarities with pilocytic

astrocytomasbull Until recently these were

grouped with PAbull These involve entire

neuraxisbull Mean age at preentation is

18 monthsbull Most are found in

hypothalamic ndash chiasmicregion

bull Pts present with features ofraised ICP

bull RAISED HEADCIRCUMFERENCE may beonly subtle sign in infants

bull Histologically consists ofmyxoid matrix with cells inloose fibrillary and myxoidbackground

bull Tumor cells formperivascular rosettes

10202016 20unusual giomas

bull Preferred sites includebull (1) optic nerve (lsquooptic

nerve gliomarsquo)bull (2) optic

chiasmhypothalamusbull (3) thalamus and basal

gangliabull (4) cerebral hemispherebull (5) cerebellum (lsquocerebellar

astrocytomarsquo) andbull (6) brainstem (dorsal

exophytic brainstemglioma)

bull Genetically chracterized bythe presence of NF1mutations BRAFduplications and theabsence of IDH1 mutation

10202016 21unusual giomas

Imaging

bull The huge chiasmatic-hypothalamic tumor revealslow signal intensity on aT1-weighted image (A)

bull Bright signal intensity on aT2-weighted image (B)

bull Gadolinium-enhancedbrain MR images showheterogeneously strongenhancement (C and D)

bull Leptomeningealdisseminations at thecervical spine levels

10202016 22unusual giomas

bull NO standard careprotocols have beendefined

bull As these are newlydefined and raretumors

bull Surgical resection isfavoured if locationfavours

bull Mean progression freesurvival is 25 months

bull Mean overall survival is60 months

bull Reduced survival isbecause of inability toachieve gross resection

10202016 23unusual giomas

bull The usual indications foradjuvant treatment(chemotherapy orradiation therapy)include tumor recurrenceafter initial completeresection or symptomatictumors

bull In addition adjuvanttreatment is generallyindicated for tumors withgrowth on follow-upimaging even in theabsence of symptoms

bull The role of chemotherapyis in evolution

bull Chemotherapy may beimplemented for the treatment ofinoperable or partially resectedgliomas

bull The decision to use postoperativeadjuvant therapy also depends ona lesions perceived malignanttendencies as the potentialbenefits of radiation therapy andchemotherapy must be weighedagainst their morbidity

bull The decision to use postoperativeadjuvant therapy also depends ona lesions perceived malignanttendencies as the potentialbenefits of radiation therapy andchemotherapy must be weighedagainst their morbidity

10202016 24unusual giomas

Pleomorphic xanthoastrocytoma

bull First described by Kepsand colleuges in 1979

bull Represents lt1 of allastrocytomas

bull May be found alongentire neuraxis mostlysupratentorial( temporallobe)

bull Cerebellum and soinalcord are the other sites

bull Pts present in 2nd and 3rd

decadesbull Median age at 14 yearsbull Both sexes are affected

equallybull 70-80 pts present with

seizures and headachefocal neuro deficits

bull Rarely tumor mayhemmorrhage

10202016 25unusual giomas

bull Supratentorail are mostoften cortical and meningesbased

bull Chronic seizuresbull Grossely these are firm

tumors variable in colorrelatively avascular

bull Typically invade the piaarachnoid space

bull 13 pts have involvementof all the three meninges

bull Cyst fluid is xanthochromic

bull Very specific microscpoiccriteria for diagnosis are

1 Pleomorphic andxanthomatous astrocytes

2 Perivascular lymphocytes3 Non infilterating histology4 Reticulin stainingbull Pleomorphism can be as

vast as glioblastoma orsarcomatous tumors

10202016 26unusual giomas

bull IHC Staining revealsspindle cells and roundpleomorphic cellspositive for S-100 andGFAP

bull Staining forsynaptophysin and NSEmay be also present

bull Some workers havereported them to be asubtype of ganglioglioma

bull Association between theNeurofibromatosis Sturge Weber Syndrome

bull Loss of chr 9 and gain ofchromosome 7 have beenfound

10202016 27unusual giomas

Imaging

bull CTbull PXAs are typically hypo

or isodense and may bewell or poorlydemarcated usually withno or little surroundingoedema

bull Calcification is rarebull Due to its superficial

location it may causescalloping of theoverlying bone

10202016 28unusual giomas

bull MRIbull T1

ndash solid component iso tohypointense cf grey matter

ndash cystic component low signalndash leptomeningeal involvement

seen in over 70 of casesbull T1 C+ (Gd)

ndash solid component usuallyenhances vividly

bull T2ndash solid component iso to

hyperintense cf grey matterndash cystic component high signalndash on T2 FLAIR sequence cystic

areas show hyperintensityrelative to CSF due to higherprotein contents

ndash little surrounding vasogenicoedema

10202016 29unusual giomas

Management amp Outcome

bull Quite rare tumorsbull Surgical excision appears to

be best favoured modalitybull Actuarial survival trates are

91 at 5 years and 82 at10 years

bull Median survival 18 years

bull Total resection should beattempted in every case asfar as possible

bull Resection of cyst lining isnot indicated

bull Data regarding the use ofadjuvant therapy at thistime are limited to few casereports only

bull Pts who had incompleteresection high mitoticindex necrosis should bereserved for strict vigilanceand CEMRI on follow ups

10202016 30unusual giomas

bull At progression repeatresection be attempted

bull Proression should betaken as aggressivetumor type

bull Malignanttransformation maynecessitate adjuvanttherapy

10202016 31unusual giomas

bull Br J Neurosurg 2013 Dec27(6)759-64 doi 103109026886972013776666 Epub 2013 Mar 20

bull Pleomorphic xanthoastrocytoma long-term results of surgical treatment and analysis of prognosticfactors

bull Gallo P1 Cecchi PC Locatelli F Rizzo P Ghimenton C Gerosa M Pinna G

bull BACKGROUND

bull Pleomorphic Xanthoastrocytoma (PXA) is a rare brain tumour most commonly affecting children andyoung adults To date only few data regarding the long-term follow-up of these patients after surgery are

available The aim of this study is to describe our single-institution experience in the surgicalmanagement of this particular glioma over a period of over 18 years

bull METHODS

bull We performed a retrospective review of all cases of PXA (40 patients) operated upon at theDepartment of Neurosurgery of Verona Italy between 1990 and 2008 The impact of clinical radiologicalsurgical and histological factors on overall survival (OS) and progression-free survival (PFS) was analysed bymeans of univariate and multivariate models

bull FINDINGS

bull We achieved a gross total resection (GTR) in 65 of patients Histological diagnosis was of grade II in 80anaplastic features were present in the remaining 20 Adjuvant treatment radiotherapy or chemo-

radiotherapy was administered in 40 of the cases Median follow-up was 74 months OS at 5- and10 years was 7632 and 6824 respectively PFS at 5- and 10 years was 71 and 58respectively In the multivariate model histological grade extent of resection and age at diagnosis (le 30years vs gt 30 years) were the only independent prognostic factors for both OS and PFS

bull CONCLUSIONS

bull Our retrospective long-term study confirms the relatively favourable prognosis associatedwith PXA Young patients with a low-grade tumour (WHO grade II) who underwent GTR carrythe longest OS and PFS

10202016 32unusual giomas

bull Brain Tumor Pathol 2006 Apr23(1)55-63

bull A case of anaplastic pleomorphic xanthoastrocytoma presenting with tumor bleeding and cerebrospinal fluid dissemination

bull Asano K1 Miyamoto S Kubo O Kikkukawa T Yagihashi A Ohkuma H

bull Pleomorphic xanthoastrocytoma (PXA) has been considered an astrocytic tumorwith a relatively favorable prognosis However PXA cases having several recurrent patterns with poor prognosis have been reported in recent years and a new concept of anaplastic PXA has been proposed The present case was a 59-year-old woman who presented with tumor bleeding onset and cerebrospinal fluid dissemination The patient had sudden-onset right hemiparesis aphasia and consciousness disturbance and was admitted to a local area hospital After emergency surgery had removed the hematoma postoperative contrast-enhanced CT scan revealed a left temporal tumor A second surgery was therefore performed for initial tumor removal 2 months later Histopathological findings showed that the tumor was typical PXA with strong pleomorphism and xanthomatous changes and contained an ependymoma-like component in the center area However endothelial proliferation and mitosis were more remarkable compared to ordinary PXA The MIB-1 labeling index was 98 high From these findings the histopathological diagnosis was anaplastic PXA The patient underwent surgery to remove recurrent tumors 5 and 16 months later The patient died 36 months after the first onset and CT revealed glioblastoma-like

findings and cerebrospinal fluid dissemination This case report is the first case in which PXA presented with tumor bleeding onset Histopathological findings suggested anaplastic PXA from the first surgical specimens and PXA recurred many times We thus believe that the patient displayed primary anaplastic PXA rather than secondary anaplastic PXA that results in malignant transformation10202016 33unusual giomas

Ganglioglioma

bull WHO grade 1bull Initially described by

Perkins in 1926bull Benign well

differentiatedneuroepithelial tumors

bull Most commonly found intemporal lobe though canoccur anywhere in CNS

bull Commonly found inyoung patients withepilepsy

bull Most commonly notedtumor in temporal lobeepilepsy patients

bull Have been described inbrain steem spinal cordand cerebellum

bull Account for 1 ofprimary brain tumors

10202016 34unusual giomas

bull Male dominencebull Most common features

are seizures headachedizziness ataxiaprogressive weakness

bull Malignanttransformation is rare

bull Histology showsimmature mixture ofabnormal neural andglial elements

bull Macroscopically appearsolid or cystic

bull Cystic are welldelineatedfromsurrounding brain

bull Calcification is common

10202016 35unusual giomas

bull IHC staing shows reactivity for GFAP S-100 andvimentin

bull Neurons show synaptophysin class III betatubulin NFP chromogranin reactivity

10202016 36unusual giomas

Imaging

bull Highly variable imagingcharacters

bull No special typicalimaging criteria

bull Enhancement patternsare highly variable

bull Iso to hypointense onT1WI

bull Heterogenous andhyperintense on T2WI

bull Can contain solid cyticcalcified components

10202016 37unusual giomas

bull FDG PET scan showshypometabolism oreumetabolism in thesepatients

10202016 38unusual giomas

Management And Outcome

bull Gold standard is grosstotal resection

bull Seizure control aftersurgery is as high as80

bull Survival rates are highbull Event free survival of

low grade and highgrade gangiogioma are57 vs 15

bull The optimal surgicalresection depends uponthe eloquent areasinvolved in the vicinityof the tumor

bull The use ofchemotherapy is notwell established

bull Typically they shouldbe allowed for chemoand radiotherapy

10202016 39unusual giomas

bull The use of adjuvant therapy is warranted in thosecases which harbour residual lesions or features ofanaplasia

bull Potential harm to nearby brain areas preclude thejudicious use of radiation

10202016 40unusual giomas

bull Brain Tumor Res Treat 2014 Oct 2(2) 49ndash55

bull Treatment and Outcomes for Gangliogliomas A Single-Center Review of 16 Patients

bull Jye Young Song Jeong Hoon Kim Young Hyun Cho Chang Jin Kim and Eun Jung Lee

bull Ganglioglioma is a rare and slowly growing benign tumor We investigated the outcomes of patients who underwent different combination treatments

bull Methods

bull Between 1998 and 2012 16 patients including 11 men and 5 women with a median age of 125 years (range 25-65 years) were treated for intracranial gangliogliomas at our institution The median follow-up period was 57 years (range 48 days-156 years) Fifteen cases were included in the outcome assessment because one patient was lost to follow-up Complete resection was achieved in 8 (53) patients Six (40) patients underwent incomplete resection with or without adjuvant radiotherapy and one patient with a brainstem tumor underwent only stereotactic biopsy

bull Results

bull Gangliogliomas predominantly affected young (875) male patients and most frequently presented with seizures (64) Of eight patients who underwent complete resection seven did not show recurrence whereas only three of six with incomplete resection showed no recurrence Four patients with recurrence received salvage treatments (two repeat surgeries and two radiosurgeries) A tumor control rate of 93 (1415) was achieved at the last follow-up No recurrence or malignant changes were observed after a median follow-up of 12 and 45 years in four patients who received gamma knife (GK) radiosurgery as adjuvant and salvage treatment

bull Conclusion

bull Complete resection produced the best outcomes and incomplete resection followed by adjuvant or salvage treatments showed favorable outcomes In patients who are not eligible for complete resection because of tumor location or potential neurologic deficits following surgery GK radiosurgery should be considered for the treatment of residual or recurrent tumors

10202016 41unusual giomas

Papillary tumours of pineal

bull WHO grade II AND IIIbull Relatively new edition to WHObull They are equal in both sexesbull Reach medical attention due to headache mental

status changes and altered visionbull Contain eosinophilic cells with distinctive

borders with large pleomorphic nucleibull Positive for cytokeratin synaptophysin stain

variably for GFAP vimentin S-100

10202016 unusual giomas 42

Imaging

bull Generally found to be diffusely enhancinglesion of pineal region

bull Post 3rd ventricle with HCPbull Appear well demarcated from thalamus and

cerebellum

10202016 unusual giomas 43

Mangement

bull Standard treatment is excisionbull Grow slowly and donot transform to

malignant tumorsbull Local recurrence is however possiblebull Radiotherapy appears to provide good control

in local recurrence

10202016 unusual giomas 44

Choroid Glioma Of Third Ventricle

bull WHO Grade IIbull Ist recognised by WHO in

1998bull Arise in the anterior third

ventriclebull Commonly come to

attention after HCPbull Appera to contain in 3rd

ventricle and thought toarise from ventricularsurface

bull Composed of GFAP andvimentin positive cells inmucinous matrix

bull Nuclei are uniform insize and shape

bull Indolent growthbull May show EGF receptor

positivitybull Some suggets these tumor

arise from the lminaterminalis andventricular tanycytes

10202016 45unusual giomas

Imaging

bull Commonly fill 3rd

ventriclebull Diffusely enhance with

contrastbull Appear to infiltrate the

thalamusbull Large tumors may be

difficult to differentiatefrom suprasellar masses

10202016 46unusual giomas

10202016 47unusual giomas

bull Surgical management is limited by locationbull Access through the dialted ventricle and lamina

terminalis may be challangingbull Subtotal resection is thus commonbull Role of adjuvant radiotherapy is unknown

10202016 48unusual giomas

bull Acta Neurochir (Wien) 2008 Nov150(11)1183-91 doi 101007s00701-008-0014-6 Epub

2008 Oct 21

bull Chordoid glioma of the third ventricle

bull Vanhauwaert DJ1 Clement F Van Dorpe J Deruytter MJKGROUND

bull Chordoid glioma is a rare tumour (World Health Organisation grade II) originating from the third ventricle

with both glial and chordoid features It was first described by Brat in 1998 Since there is no detailed

information available on the outcome after surgery and adjuvant treatment we reviewed the literature

bull METHODS

bull A literature search through PUBMED revealed 50 cases of chordoid glioma Most reports

were found in pathology journals Information on the postoperative course was sometimes

very limited We reviewed the available literature and studied in detail the presenting

symptoms mortality and postoperative complications in relation to the extent of resective

surgery as well as the importance of adjuvant treatment

bull CONCLUSIONS

bull Mortality in the immediate postoperative period is 32 and is higher after gross total

resection as compared to subtotal resection Non-fatal postoperative complications are

hypothalamic disorders and mental alterations Gross total resection is the treatment of

choice since no recurrence has been reported after macroscopically complete resection

but this is often difficult because of the location and adherence to the hypothalamus The

role of postoperative radiotherapy is uncertain There is some indication that radiosurgery

with or without conventional irradiation is superior to conventional radiation alone Planned

subtotal resection followed by stereotactic radiosurgery can be a safe and effective

alternative in a patient in whom gross total resection is considered to be too risky

There is no report on the use of chemotherapy in the treatment of chordoid gliomas More

information about the optimal treatment strategy is needed and more reports are also needed10202016 49unusual giomas

bull Neurologist 2011 Jan17(1)52bull Chordoid glioma a case report and literature reviewbull Liu WP1 Cheng JX Yi XC Zhen HN Fei Z Li Q Zhang Xbull chordoid glioma is a rare tumor (World Health Organization grade II) with both

glial and chordoid features often located in the suprasellar region and anterior third ventricle It was first described by Brat in 1998 Because there is no detailed information available from the clinical perspective we reviewed the literature

bull METHODS

bull a literature search through PUBMED and CNKI revealed 64 cases of chordoid glioma Information on the clinical course was very limited We reviewed the literature and studied the pathologic and imaging features postoperative mortality and morbidity in relation to surgical extension and approaches and the importance of adjuvant treatment

bull CONCLUSIONS bull mortality in the immediate postoperative period is 28 and postoperative

morbidity is 60 which are statistically higher after gross total resection as compared with subtotal resection Translamina terminalis approach is considered to be the best approach The current study cannot document that patients have longer survival and higher quality of life after gross total resection than subtotal resection The role of postoperative radiotherapy is uncertain and there is no report on the use of chemotherapy More information about the optimal treatment strategy is needed

10202016 50unusual giomas

Conclusion

bull They are identified by their unique morphologicalfeatures

bull Many of these have indolent course and identified byepilepsy

bull Potentail diagnosis should be considered in youngpatients with seizures and cortically based lesions

bull Correct diagnosis requires experiencedneuropathologisrt

bull As more experienced is gained more knowledgetowards better understanding shall provide betteroutcomes in these patients

10202016 unusual giomas 51

10202016 52unusual giomas

Content supportndash youmann neurosurgery textbook

Pilomyxoid astrocytoma

bull Recently described tumorsbull Similarities with pilocytic

astrocytomasbull Until recently these were

grouped with PAbull These involve entire

neuraxisbull Mean age at preentation is

18 monthsbull Most are found in

hypothalamic ndash chiasmicregion

bull Pts present with features ofraised ICP

bull RAISED HEADCIRCUMFERENCE may beonly subtle sign in infants

bull Histologically consists ofmyxoid matrix with cells inloose fibrillary and myxoidbackground

bull Tumor cells formperivascular rosettes

10202016 20unusual giomas

bull Preferred sites includebull (1) optic nerve (lsquooptic

nerve gliomarsquo)bull (2) optic

chiasmhypothalamusbull (3) thalamus and basal

gangliabull (4) cerebral hemispherebull (5) cerebellum (lsquocerebellar

astrocytomarsquo) andbull (6) brainstem (dorsal

exophytic brainstemglioma)

bull Genetically chracterized bythe presence of NF1mutations BRAFduplications and theabsence of IDH1 mutation

10202016 21unusual giomas

Imaging

bull The huge chiasmatic-hypothalamic tumor revealslow signal intensity on aT1-weighted image (A)

bull Bright signal intensity on aT2-weighted image (B)

bull Gadolinium-enhancedbrain MR images showheterogeneously strongenhancement (C and D)

bull Leptomeningealdisseminations at thecervical spine levels

10202016 22unusual giomas

bull NO standard careprotocols have beendefined

bull As these are newlydefined and raretumors

bull Surgical resection isfavoured if locationfavours

bull Mean progression freesurvival is 25 months

bull Mean overall survival is60 months

bull Reduced survival isbecause of inability toachieve gross resection

10202016 23unusual giomas

bull The usual indications foradjuvant treatment(chemotherapy orradiation therapy)include tumor recurrenceafter initial completeresection or symptomatictumors

bull In addition adjuvanttreatment is generallyindicated for tumors withgrowth on follow-upimaging even in theabsence of symptoms

bull The role of chemotherapyis in evolution

bull Chemotherapy may beimplemented for the treatment ofinoperable or partially resectedgliomas

bull The decision to use postoperativeadjuvant therapy also depends ona lesions perceived malignanttendencies as the potentialbenefits of radiation therapy andchemotherapy must be weighedagainst their morbidity

bull The decision to use postoperativeadjuvant therapy also depends ona lesions perceived malignanttendencies as the potentialbenefits of radiation therapy andchemotherapy must be weighedagainst their morbidity

10202016 24unusual giomas

Pleomorphic xanthoastrocytoma

bull First described by Kepsand colleuges in 1979

bull Represents lt1 of allastrocytomas

bull May be found alongentire neuraxis mostlysupratentorial( temporallobe)

bull Cerebellum and soinalcord are the other sites

bull Pts present in 2nd and 3rd

decadesbull Median age at 14 yearsbull Both sexes are affected

equallybull 70-80 pts present with

seizures and headachefocal neuro deficits

bull Rarely tumor mayhemmorrhage

10202016 25unusual giomas

bull Supratentorail are mostoften cortical and meningesbased

bull Chronic seizuresbull Grossely these are firm

tumors variable in colorrelatively avascular

bull Typically invade the piaarachnoid space

bull 13 pts have involvementof all the three meninges

bull Cyst fluid is xanthochromic

bull Very specific microscpoiccriteria for diagnosis are

1 Pleomorphic andxanthomatous astrocytes

2 Perivascular lymphocytes3 Non infilterating histology4 Reticulin stainingbull Pleomorphism can be as

vast as glioblastoma orsarcomatous tumors

10202016 26unusual giomas

bull IHC Staining revealsspindle cells and roundpleomorphic cellspositive for S-100 andGFAP

bull Staining forsynaptophysin and NSEmay be also present

bull Some workers havereported them to be asubtype of ganglioglioma

bull Association between theNeurofibromatosis Sturge Weber Syndrome

bull Loss of chr 9 and gain ofchromosome 7 have beenfound

10202016 27unusual giomas

Imaging

bull CTbull PXAs are typically hypo

or isodense and may bewell or poorlydemarcated usually withno or little surroundingoedema

bull Calcification is rarebull Due to its superficial

location it may causescalloping of theoverlying bone

10202016 28unusual giomas

bull MRIbull T1

ndash solid component iso tohypointense cf grey matter

ndash cystic component low signalndash leptomeningeal involvement

seen in over 70 of casesbull T1 C+ (Gd)

ndash solid component usuallyenhances vividly

bull T2ndash solid component iso to

hyperintense cf grey matterndash cystic component high signalndash on T2 FLAIR sequence cystic

areas show hyperintensityrelative to CSF due to higherprotein contents

ndash little surrounding vasogenicoedema

10202016 29unusual giomas

Management amp Outcome

bull Quite rare tumorsbull Surgical excision appears to

be best favoured modalitybull Actuarial survival trates are

91 at 5 years and 82 at10 years

bull Median survival 18 years

bull Total resection should beattempted in every case asfar as possible

bull Resection of cyst lining isnot indicated

bull Data regarding the use ofadjuvant therapy at thistime are limited to few casereports only

bull Pts who had incompleteresection high mitoticindex necrosis should bereserved for strict vigilanceand CEMRI on follow ups

10202016 30unusual giomas

bull At progression repeatresection be attempted

bull Proression should betaken as aggressivetumor type

bull Malignanttransformation maynecessitate adjuvanttherapy

10202016 31unusual giomas

bull Br J Neurosurg 2013 Dec27(6)759-64 doi 103109026886972013776666 Epub 2013 Mar 20

bull Pleomorphic xanthoastrocytoma long-term results of surgical treatment and analysis of prognosticfactors

bull Gallo P1 Cecchi PC Locatelli F Rizzo P Ghimenton C Gerosa M Pinna G

bull BACKGROUND

bull Pleomorphic Xanthoastrocytoma (PXA) is a rare brain tumour most commonly affecting children andyoung adults To date only few data regarding the long-term follow-up of these patients after surgery are

available The aim of this study is to describe our single-institution experience in the surgicalmanagement of this particular glioma over a period of over 18 years

bull METHODS

bull We performed a retrospective review of all cases of PXA (40 patients) operated upon at theDepartment of Neurosurgery of Verona Italy between 1990 and 2008 The impact of clinical radiologicalsurgical and histological factors on overall survival (OS) and progression-free survival (PFS) was analysed bymeans of univariate and multivariate models

bull FINDINGS

bull We achieved a gross total resection (GTR) in 65 of patients Histological diagnosis was of grade II in 80anaplastic features were present in the remaining 20 Adjuvant treatment radiotherapy or chemo-

radiotherapy was administered in 40 of the cases Median follow-up was 74 months OS at 5- and10 years was 7632 and 6824 respectively PFS at 5- and 10 years was 71 and 58respectively In the multivariate model histological grade extent of resection and age at diagnosis (le 30years vs gt 30 years) were the only independent prognostic factors for both OS and PFS

bull CONCLUSIONS

bull Our retrospective long-term study confirms the relatively favourable prognosis associatedwith PXA Young patients with a low-grade tumour (WHO grade II) who underwent GTR carrythe longest OS and PFS

10202016 32unusual giomas

bull Brain Tumor Pathol 2006 Apr23(1)55-63

bull A case of anaplastic pleomorphic xanthoastrocytoma presenting with tumor bleeding and cerebrospinal fluid dissemination

bull Asano K1 Miyamoto S Kubo O Kikkukawa T Yagihashi A Ohkuma H

bull Pleomorphic xanthoastrocytoma (PXA) has been considered an astrocytic tumorwith a relatively favorable prognosis However PXA cases having several recurrent patterns with poor prognosis have been reported in recent years and a new concept of anaplastic PXA has been proposed The present case was a 59-year-old woman who presented with tumor bleeding onset and cerebrospinal fluid dissemination The patient had sudden-onset right hemiparesis aphasia and consciousness disturbance and was admitted to a local area hospital After emergency surgery had removed the hematoma postoperative contrast-enhanced CT scan revealed a left temporal tumor A second surgery was therefore performed for initial tumor removal 2 months later Histopathological findings showed that the tumor was typical PXA with strong pleomorphism and xanthomatous changes and contained an ependymoma-like component in the center area However endothelial proliferation and mitosis were more remarkable compared to ordinary PXA The MIB-1 labeling index was 98 high From these findings the histopathological diagnosis was anaplastic PXA The patient underwent surgery to remove recurrent tumors 5 and 16 months later The patient died 36 months after the first onset and CT revealed glioblastoma-like

findings and cerebrospinal fluid dissemination This case report is the first case in which PXA presented with tumor bleeding onset Histopathological findings suggested anaplastic PXA from the first surgical specimens and PXA recurred many times We thus believe that the patient displayed primary anaplastic PXA rather than secondary anaplastic PXA that results in malignant transformation10202016 33unusual giomas

Ganglioglioma

bull WHO grade 1bull Initially described by

Perkins in 1926bull Benign well

differentiatedneuroepithelial tumors

bull Most commonly found intemporal lobe though canoccur anywhere in CNS

bull Commonly found inyoung patients withepilepsy

bull Most commonly notedtumor in temporal lobeepilepsy patients

bull Have been described inbrain steem spinal cordand cerebellum

bull Account for 1 ofprimary brain tumors

10202016 34unusual giomas

bull Male dominencebull Most common features

are seizures headachedizziness ataxiaprogressive weakness

bull Malignanttransformation is rare

bull Histology showsimmature mixture ofabnormal neural andglial elements

bull Macroscopically appearsolid or cystic

bull Cystic are welldelineatedfromsurrounding brain

bull Calcification is common

10202016 35unusual giomas

bull IHC staing shows reactivity for GFAP S-100 andvimentin

bull Neurons show synaptophysin class III betatubulin NFP chromogranin reactivity

10202016 36unusual giomas

Imaging

bull Highly variable imagingcharacters

bull No special typicalimaging criteria

bull Enhancement patternsare highly variable

bull Iso to hypointense onT1WI

bull Heterogenous andhyperintense on T2WI

bull Can contain solid cyticcalcified components

10202016 37unusual giomas

bull FDG PET scan showshypometabolism oreumetabolism in thesepatients

10202016 38unusual giomas

Management And Outcome

bull Gold standard is grosstotal resection

bull Seizure control aftersurgery is as high as80

bull Survival rates are highbull Event free survival of

low grade and highgrade gangiogioma are57 vs 15

bull The optimal surgicalresection depends uponthe eloquent areasinvolved in the vicinityof the tumor

bull The use ofchemotherapy is notwell established

bull Typically they shouldbe allowed for chemoand radiotherapy

10202016 39unusual giomas

bull The use of adjuvant therapy is warranted in thosecases which harbour residual lesions or features ofanaplasia

bull Potential harm to nearby brain areas preclude thejudicious use of radiation

10202016 40unusual giomas

bull Brain Tumor Res Treat 2014 Oct 2(2) 49ndash55

bull Treatment and Outcomes for Gangliogliomas A Single-Center Review of 16 Patients

bull Jye Young Song Jeong Hoon Kim Young Hyun Cho Chang Jin Kim and Eun Jung Lee

bull Ganglioglioma is a rare and slowly growing benign tumor We investigated the outcomes of patients who underwent different combination treatments

bull Methods

bull Between 1998 and 2012 16 patients including 11 men and 5 women with a median age of 125 years (range 25-65 years) were treated for intracranial gangliogliomas at our institution The median follow-up period was 57 years (range 48 days-156 years) Fifteen cases were included in the outcome assessment because one patient was lost to follow-up Complete resection was achieved in 8 (53) patients Six (40) patients underwent incomplete resection with or without adjuvant radiotherapy and one patient with a brainstem tumor underwent only stereotactic biopsy

bull Results

bull Gangliogliomas predominantly affected young (875) male patients and most frequently presented with seizures (64) Of eight patients who underwent complete resection seven did not show recurrence whereas only three of six with incomplete resection showed no recurrence Four patients with recurrence received salvage treatments (two repeat surgeries and two radiosurgeries) A tumor control rate of 93 (1415) was achieved at the last follow-up No recurrence or malignant changes were observed after a median follow-up of 12 and 45 years in four patients who received gamma knife (GK) radiosurgery as adjuvant and salvage treatment

bull Conclusion

bull Complete resection produced the best outcomes and incomplete resection followed by adjuvant or salvage treatments showed favorable outcomes In patients who are not eligible for complete resection because of tumor location or potential neurologic deficits following surgery GK radiosurgery should be considered for the treatment of residual or recurrent tumors

10202016 41unusual giomas

Papillary tumours of pineal

bull WHO grade II AND IIIbull Relatively new edition to WHObull They are equal in both sexesbull Reach medical attention due to headache mental

status changes and altered visionbull Contain eosinophilic cells with distinctive

borders with large pleomorphic nucleibull Positive for cytokeratin synaptophysin stain

variably for GFAP vimentin S-100

10202016 unusual giomas 42

Imaging

bull Generally found to be diffusely enhancinglesion of pineal region

bull Post 3rd ventricle with HCPbull Appear well demarcated from thalamus and

cerebellum

10202016 unusual giomas 43

Mangement

bull Standard treatment is excisionbull Grow slowly and donot transform to

malignant tumorsbull Local recurrence is however possiblebull Radiotherapy appears to provide good control

in local recurrence

10202016 unusual giomas 44

Choroid Glioma Of Third Ventricle

bull WHO Grade IIbull Ist recognised by WHO in

1998bull Arise in the anterior third

ventriclebull Commonly come to

attention after HCPbull Appera to contain in 3rd

ventricle and thought toarise from ventricularsurface

bull Composed of GFAP andvimentin positive cells inmucinous matrix

bull Nuclei are uniform insize and shape

bull Indolent growthbull May show EGF receptor

positivitybull Some suggets these tumor

arise from the lminaterminalis andventricular tanycytes

10202016 45unusual giomas

Imaging

bull Commonly fill 3rd

ventriclebull Diffusely enhance with

contrastbull Appear to infiltrate the

thalamusbull Large tumors may be

difficult to differentiatefrom suprasellar masses

10202016 46unusual giomas

10202016 47unusual giomas

bull Surgical management is limited by locationbull Access through the dialted ventricle and lamina

terminalis may be challangingbull Subtotal resection is thus commonbull Role of adjuvant radiotherapy is unknown

10202016 48unusual giomas

bull Acta Neurochir (Wien) 2008 Nov150(11)1183-91 doi 101007s00701-008-0014-6 Epub

2008 Oct 21

bull Chordoid glioma of the third ventricle

bull Vanhauwaert DJ1 Clement F Van Dorpe J Deruytter MJKGROUND

bull Chordoid glioma is a rare tumour (World Health Organisation grade II) originating from the third ventricle

with both glial and chordoid features It was first described by Brat in 1998 Since there is no detailed

information available on the outcome after surgery and adjuvant treatment we reviewed the literature

bull METHODS

bull A literature search through PUBMED revealed 50 cases of chordoid glioma Most reports

were found in pathology journals Information on the postoperative course was sometimes

very limited We reviewed the available literature and studied in detail the presenting

symptoms mortality and postoperative complications in relation to the extent of resective

surgery as well as the importance of adjuvant treatment

bull CONCLUSIONS

bull Mortality in the immediate postoperative period is 32 and is higher after gross total

resection as compared to subtotal resection Non-fatal postoperative complications are

hypothalamic disorders and mental alterations Gross total resection is the treatment of

choice since no recurrence has been reported after macroscopically complete resection

but this is often difficult because of the location and adherence to the hypothalamus The

role of postoperative radiotherapy is uncertain There is some indication that radiosurgery

with or without conventional irradiation is superior to conventional radiation alone Planned

subtotal resection followed by stereotactic radiosurgery can be a safe and effective

alternative in a patient in whom gross total resection is considered to be too risky

There is no report on the use of chemotherapy in the treatment of chordoid gliomas More

information about the optimal treatment strategy is needed and more reports are also needed10202016 49unusual giomas

bull Neurologist 2011 Jan17(1)52bull Chordoid glioma a case report and literature reviewbull Liu WP1 Cheng JX Yi XC Zhen HN Fei Z Li Q Zhang Xbull chordoid glioma is a rare tumor (World Health Organization grade II) with both

glial and chordoid features often located in the suprasellar region and anterior third ventricle It was first described by Brat in 1998 Because there is no detailed information available from the clinical perspective we reviewed the literature

bull METHODS

bull a literature search through PUBMED and CNKI revealed 64 cases of chordoid glioma Information on the clinical course was very limited We reviewed the literature and studied the pathologic and imaging features postoperative mortality and morbidity in relation to surgical extension and approaches and the importance of adjuvant treatment

bull CONCLUSIONS bull mortality in the immediate postoperative period is 28 and postoperative

morbidity is 60 which are statistically higher after gross total resection as compared with subtotal resection Translamina terminalis approach is considered to be the best approach The current study cannot document that patients have longer survival and higher quality of life after gross total resection than subtotal resection The role of postoperative radiotherapy is uncertain and there is no report on the use of chemotherapy More information about the optimal treatment strategy is needed

10202016 50unusual giomas

Conclusion

bull They are identified by their unique morphologicalfeatures

bull Many of these have indolent course and identified byepilepsy

bull Potentail diagnosis should be considered in youngpatients with seizures and cortically based lesions

bull Correct diagnosis requires experiencedneuropathologisrt

bull As more experienced is gained more knowledgetowards better understanding shall provide betteroutcomes in these patients

10202016 unusual giomas 51

10202016 52unusual giomas

Content supportndash youmann neurosurgery textbook

bull Preferred sites includebull (1) optic nerve (lsquooptic

nerve gliomarsquo)bull (2) optic

chiasmhypothalamusbull (3) thalamus and basal

gangliabull (4) cerebral hemispherebull (5) cerebellum (lsquocerebellar

astrocytomarsquo) andbull (6) brainstem (dorsal

exophytic brainstemglioma)

bull Genetically chracterized bythe presence of NF1mutations BRAFduplications and theabsence of IDH1 mutation

10202016 21unusual giomas

Imaging

bull The huge chiasmatic-hypothalamic tumor revealslow signal intensity on aT1-weighted image (A)

bull Bright signal intensity on aT2-weighted image (B)

bull Gadolinium-enhancedbrain MR images showheterogeneously strongenhancement (C and D)

bull Leptomeningealdisseminations at thecervical spine levels

10202016 22unusual giomas

bull NO standard careprotocols have beendefined

bull As these are newlydefined and raretumors

bull Surgical resection isfavoured if locationfavours

bull Mean progression freesurvival is 25 months

bull Mean overall survival is60 months

bull Reduced survival isbecause of inability toachieve gross resection

10202016 23unusual giomas

bull The usual indications foradjuvant treatment(chemotherapy orradiation therapy)include tumor recurrenceafter initial completeresection or symptomatictumors

bull In addition adjuvanttreatment is generallyindicated for tumors withgrowth on follow-upimaging even in theabsence of symptoms

bull The role of chemotherapyis in evolution

bull Chemotherapy may beimplemented for the treatment ofinoperable or partially resectedgliomas

bull The decision to use postoperativeadjuvant therapy also depends ona lesions perceived malignanttendencies as the potentialbenefits of radiation therapy andchemotherapy must be weighedagainst their morbidity

bull The decision to use postoperativeadjuvant therapy also depends ona lesions perceived malignanttendencies as the potentialbenefits of radiation therapy andchemotherapy must be weighedagainst their morbidity

10202016 24unusual giomas

Pleomorphic xanthoastrocytoma

bull First described by Kepsand colleuges in 1979

bull Represents lt1 of allastrocytomas

bull May be found alongentire neuraxis mostlysupratentorial( temporallobe)

bull Cerebellum and soinalcord are the other sites

bull Pts present in 2nd and 3rd

decadesbull Median age at 14 yearsbull Both sexes are affected

equallybull 70-80 pts present with

seizures and headachefocal neuro deficits

bull Rarely tumor mayhemmorrhage

10202016 25unusual giomas

bull Supratentorail are mostoften cortical and meningesbased

bull Chronic seizuresbull Grossely these are firm

tumors variable in colorrelatively avascular

bull Typically invade the piaarachnoid space

bull 13 pts have involvementof all the three meninges

bull Cyst fluid is xanthochromic

bull Very specific microscpoiccriteria for diagnosis are

1 Pleomorphic andxanthomatous astrocytes

2 Perivascular lymphocytes3 Non infilterating histology4 Reticulin stainingbull Pleomorphism can be as

vast as glioblastoma orsarcomatous tumors

10202016 26unusual giomas

bull IHC Staining revealsspindle cells and roundpleomorphic cellspositive for S-100 andGFAP

bull Staining forsynaptophysin and NSEmay be also present

bull Some workers havereported them to be asubtype of ganglioglioma

bull Association between theNeurofibromatosis Sturge Weber Syndrome

bull Loss of chr 9 and gain ofchromosome 7 have beenfound

10202016 27unusual giomas

Imaging

bull CTbull PXAs are typically hypo

or isodense and may bewell or poorlydemarcated usually withno or little surroundingoedema

bull Calcification is rarebull Due to its superficial

location it may causescalloping of theoverlying bone

10202016 28unusual giomas

bull MRIbull T1

ndash solid component iso tohypointense cf grey matter

ndash cystic component low signalndash leptomeningeal involvement

seen in over 70 of casesbull T1 C+ (Gd)

ndash solid component usuallyenhances vividly

bull T2ndash solid component iso to

hyperintense cf grey matterndash cystic component high signalndash on T2 FLAIR sequence cystic

areas show hyperintensityrelative to CSF due to higherprotein contents

ndash little surrounding vasogenicoedema

10202016 29unusual giomas

Management amp Outcome

bull Quite rare tumorsbull Surgical excision appears to

be best favoured modalitybull Actuarial survival trates are

91 at 5 years and 82 at10 years

bull Median survival 18 years

bull Total resection should beattempted in every case asfar as possible

bull Resection of cyst lining isnot indicated

bull Data regarding the use ofadjuvant therapy at thistime are limited to few casereports only

bull Pts who had incompleteresection high mitoticindex necrosis should bereserved for strict vigilanceand CEMRI on follow ups

10202016 30unusual giomas

bull At progression repeatresection be attempted

bull Proression should betaken as aggressivetumor type

bull Malignanttransformation maynecessitate adjuvanttherapy

10202016 31unusual giomas

bull Br J Neurosurg 2013 Dec27(6)759-64 doi 103109026886972013776666 Epub 2013 Mar 20

bull Pleomorphic xanthoastrocytoma long-term results of surgical treatment and analysis of prognosticfactors

bull Gallo P1 Cecchi PC Locatelli F Rizzo P Ghimenton C Gerosa M Pinna G

bull BACKGROUND

bull Pleomorphic Xanthoastrocytoma (PXA) is a rare brain tumour most commonly affecting children andyoung adults To date only few data regarding the long-term follow-up of these patients after surgery are

available The aim of this study is to describe our single-institution experience in the surgicalmanagement of this particular glioma over a period of over 18 years

bull METHODS

bull We performed a retrospective review of all cases of PXA (40 patients) operated upon at theDepartment of Neurosurgery of Verona Italy between 1990 and 2008 The impact of clinical radiologicalsurgical and histological factors on overall survival (OS) and progression-free survival (PFS) was analysed bymeans of univariate and multivariate models

bull FINDINGS

bull We achieved a gross total resection (GTR) in 65 of patients Histological diagnosis was of grade II in 80anaplastic features were present in the remaining 20 Adjuvant treatment radiotherapy or chemo-

radiotherapy was administered in 40 of the cases Median follow-up was 74 months OS at 5- and10 years was 7632 and 6824 respectively PFS at 5- and 10 years was 71 and 58respectively In the multivariate model histological grade extent of resection and age at diagnosis (le 30years vs gt 30 years) were the only independent prognostic factors for both OS and PFS

bull CONCLUSIONS

bull Our retrospective long-term study confirms the relatively favourable prognosis associatedwith PXA Young patients with a low-grade tumour (WHO grade II) who underwent GTR carrythe longest OS and PFS

10202016 32unusual giomas

bull Brain Tumor Pathol 2006 Apr23(1)55-63

bull A case of anaplastic pleomorphic xanthoastrocytoma presenting with tumor bleeding and cerebrospinal fluid dissemination

bull Asano K1 Miyamoto S Kubo O Kikkukawa T Yagihashi A Ohkuma H

bull Pleomorphic xanthoastrocytoma (PXA) has been considered an astrocytic tumorwith a relatively favorable prognosis However PXA cases having several recurrent patterns with poor prognosis have been reported in recent years and a new concept of anaplastic PXA has been proposed The present case was a 59-year-old woman who presented with tumor bleeding onset and cerebrospinal fluid dissemination The patient had sudden-onset right hemiparesis aphasia and consciousness disturbance and was admitted to a local area hospital After emergency surgery had removed the hematoma postoperative contrast-enhanced CT scan revealed a left temporal tumor A second surgery was therefore performed for initial tumor removal 2 months later Histopathological findings showed that the tumor was typical PXA with strong pleomorphism and xanthomatous changes and contained an ependymoma-like component in the center area However endothelial proliferation and mitosis were more remarkable compared to ordinary PXA The MIB-1 labeling index was 98 high From these findings the histopathological diagnosis was anaplastic PXA The patient underwent surgery to remove recurrent tumors 5 and 16 months later The patient died 36 months after the first onset and CT revealed glioblastoma-like

findings and cerebrospinal fluid dissemination This case report is the first case in which PXA presented with tumor bleeding onset Histopathological findings suggested anaplastic PXA from the first surgical specimens and PXA recurred many times We thus believe that the patient displayed primary anaplastic PXA rather than secondary anaplastic PXA that results in malignant transformation10202016 33unusual giomas

Ganglioglioma

bull WHO grade 1bull Initially described by

Perkins in 1926bull Benign well

differentiatedneuroepithelial tumors

bull Most commonly found intemporal lobe though canoccur anywhere in CNS

bull Commonly found inyoung patients withepilepsy

bull Most commonly notedtumor in temporal lobeepilepsy patients

bull Have been described inbrain steem spinal cordand cerebellum

bull Account for 1 ofprimary brain tumors

10202016 34unusual giomas

bull Male dominencebull Most common features

are seizures headachedizziness ataxiaprogressive weakness

bull Malignanttransformation is rare

bull Histology showsimmature mixture ofabnormal neural andglial elements

bull Macroscopically appearsolid or cystic

bull Cystic are welldelineatedfromsurrounding brain

bull Calcification is common

10202016 35unusual giomas

bull IHC staing shows reactivity for GFAP S-100 andvimentin

bull Neurons show synaptophysin class III betatubulin NFP chromogranin reactivity

10202016 36unusual giomas

Imaging

bull Highly variable imagingcharacters

bull No special typicalimaging criteria

bull Enhancement patternsare highly variable

bull Iso to hypointense onT1WI

bull Heterogenous andhyperintense on T2WI

bull Can contain solid cyticcalcified components

10202016 37unusual giomas

bull FDG PET scan showshypometabolism oreumetabolism in thesepatients

10202016 38unusual giomas

Management And Outcome

bull Gold standard is grosstotal resection

bull Seizure control aftersurgery is as high as80

bull Survival rates are highbull Event free survival of

low grade and highgrade gangiogioma are57 vs 15

bull The optimal surgicalresection depends uponthe eloquent areasinvolved in the vicinityof the tumor

bull The use ofchemotherapy is notwell established

bull Typically they shouldbe allowed for chemoand radiotherapy

10202016 39unusual giomas

bull The use of adjuvant therapy is warranted in thosecases which harbour residual lesions or features ofanaplasia

bull Potential harm to nearby brain areas preclude thejudicious use of radiation

10202016 40unusual giomas

bull Brain Tumor Res Treat 2014 Oct 2(2) 49ndash55

bull Treatment and Outcomes for Gangliogliomas A Single-Center Review of 16 Patients

bull Jye Young Song Jeong Hoon Kim Young Hyun Cho Chang Jin Kim and Eun Jung Lee

bull Ganglioglioma is a rare and slowly growing benign tumor We investigated the outcomes of patients who underwent different combination treatments

bull Methods

bull Between 1998 and 2012 16 patients including 11 men and 5 women with a median age of 125 years (range 25-65 years) were treated for intracranial gangliogliomas at our institution The median follow-up period was 57 years (range 48 days-156 years) Fifteen cases were included in the outcome assessment because one patient was lost to follow-up Complete resection was achieved in 8 (53) patients Six (40) patients underwent incomplete resection with or without adjuvant radiotherapy and one patient with a brainstem tumor underwent only stereotactic biopsy

bull Results

bull Gangliogliomas predominantly affected young (875) male patients and most frequently presented with seizures (64) Of eight patients who underwent complete resection seven did not show recurrence whereas only three of six with incomplete resection showed no recurrence Four patients with recurrence received salvage treatments (two repeat surgeries and two radiosurgeries) A tumor control rate of 93 (1415) was achieved at the last follow-up No recurrence or malignant changes were observed after a median follow-up of 12 and 45 years in four patients who received gamma knife (GK) radiosurgery as adjuvant and salvage treatment

bull Conclusion

bull Complete resection produced the best outcomes and incomplete resection followed by adjuvant or salvage treatments showed favorable outcomes In patients who are not eligible for complete resection because of tumor location or potential neurologic deficits following surgery GK radiosurgery should be considered for the treatment of residual or recurrent tumors

10202016 41unusual giomas

Papillary tumours of pineal

bull WHO grade II AND IIIbull Relatively new edition to WHObull They are equal in both sexesbull Reach medical attention due to headache mental

status changes and altered visionbull Contain eosinophilic cells with distinctive

borders with large pleomorphic nucleibull Positive for cytokeratin synaptophysin stain

variably for GFAP vimentin S-100

10202016 unusual giomas 42

Imaging

bull Generally found to be diffusely enhancinglesion of pineal region

bull Post 3rd ventricle with HCPbull Appear well demarcated from thalamus and

cerebellum

10202016 unusual giomas 43

Mangement

bull Standard treatment is excisionbull Grow slowly and donot transform to

malignant tumorsbull Local recurrence is however possiblebull Radiotherapy appears to provide good control

in local recurrence

10202016 unusual giomas 44

Choroid Glioma Of Third Ventricle

bull WHO Grade IIbull Ist recognised by WHO in

1998bull Arise in the anterior third

ventriclebull Commonly come to

attention after HCPbull Appera to contain in 3rd

ventricle and thought toarise from ventricularsurface

bull Composed of GFAP andvimentin positive cells inmucinous matrix

bull Nuclei are uniform insize and shape

bull Indolent growthbull May show EGF receptor

positivitybull Some suggets these tumor

arise from the lminaterminalis andventricular tanycytes

10202016 45unusual giomas

Imaging

bull Commonly fill 3rd

ventriclebull Diffusely enhance with

contrastbull Appear to infiltrate the

thalamusbull Large tumors may be

difficult to differentiatefrom suprasellar masses

10202016 46unusual giomas

10202016 47unusual giomas

bull Surgical management is limited by locationbull Access through the dialted ventricle and lamina

terminalis may be challangingbull Subtotal resection is thus commonbull Role of adjuvant radiotherapy is unknown

10202016 48unusual giomas

bull Acta Neurochir (Wien) 2008 Nov150(11)1183-91 doi 101007s00701-008-0014-6 Epub

2008 Oct 21

bull Chordoid glioma of the third ventricle

bull Vanhauwaert DJ1 Clement F Van Dorpe J Deruytter MJKGROUND

bull Chordoid glioma is a rare tumour (World Health Organisation grade II) originating from the third ventricle

with both glial and chordoid features It was first described by Brat in 1998 Since there is no detailed

information available on the outcome after surgery and adjuvant treatment we reviewed the literature

bull METHODS

bull A literature search through PUBMED revealed 50 cases of chordoid glioma Most reports

were found in pathology journals Information on the postoperative course was sometimes

very limited We reviewed the available literature and studied in detail the presenting

symptoms mortality and postoperative complications in relation to the extent of resective

surgery as well as the importance of adjuvant treatment

bull CONCLUSIONS

bull Mortality in the immediate postoperative period is 32 and is higher after gross total

resection as compared to subtotal resection Non-fatal postoperative complications are

hypothalamic disorders and mental alterations Gross total resection is the treatment of

choice since no recurrence has been reported after macroscopically complete resection

but this is often difficult because of the location and adherence to the hypothalamus The

role of postoperative radiotherapy is uncertain There is some indication that radiosurgery

with or without conventional irradiation is superior to conventional radiation alone Planned

subtotal resection followed by stereotactic radiosurgery can be a safe and effective

alternative in a patient in whom gross total resection is considered to be too risky

There is no report on the use of chemotherapy in the treatment of chordoid gliomas More

information about the optimal treatment strategy is needed and more reports are also needed10202016 49unusual giomas

bull Neurologist 2011 Jan17(1)52bull Chordoid glioma a case report and literature reviewbull Liu WP1 Cheng JX Yi XC Zhen HN Fei Z Li Q Zhang Xbull chordoid glioma is a rare tumor (World Health Organization grade II) with both

glial and chordoid features often located in the suprasellar region and anterior third ventricle It was first described by Brat in 1998 Because there is no detailed information available from the clinical perspective we reviewed the literature

bull METHODS

bull a literature search through PUBMED and CNKI revealed 64 cases of chordoid glioma Information on the clinical course was very limited We reviewed the literature and studied the pathologic and imaging features postoperative mortality and morbidity in relation to surgical extension and approaches and the importance of adjuvant treatment

bull CONCLUSIONS bull mortality in the immediate postoperative period is 28 and postoperative

morbidity is 60 which are statistically higher after gross total resection as compared with subtotal resection Translamina terminalis approach is considered to be the best approach The current study cannot document that patients have longer survival and higher quality of life after gross total resection than subtotal resection The role of postoperative radiotherapy is uncertain and there is no report on the use of chemotherapy More information about the optimal treatment strategy is needed

10202016 50unusual giomas

Conclusion

bull They are identified by their unique morphologicalfeatures

bull Many of these have indolent course and identified byepilepsy

bull Potentail diagnosis should be considered in youngpatients with seizures and cortically based lesions

bull Correct diagnosis requires experiencedneuropathologisrt

bull As more experienced is gained more knowledgetowards better understanding shall provide betteroutcomes in these patients

10202016 unusual giomas 51

10202016 52unusual giomas

Content supportndash youmann neurosurgery textbook

Imaging

bull The huge chiasmatic-hypothalamic tumor revealslow signal intensity on aT1-weighted image (A)

bull Bright signal intensity on aT2-weighted image (B)

bull Gadolinium-enhancedbrain MR images showheterogeneously strongenhancement (C and D)

bull Leptomeningealdisseminations at thecervical spine levels

10202016 22unusual giomas

bull NO standard careprotocols have beendefined

bull As these are newlydefined and raretumors

bull Surgical resection isfavoured if locationfavours

bull Mean progression freesurvival is 25 months

bull Mean overall survival is60 months

bull Reduced survival isbecause of inability toachieve gross resection

10202016 23unusual giomas

bull The usual indications foradjuvant treatment(chemotherapy orradiation therapy)include tumor recurrenceafter initial completeresection or symptomatictumors

bull In addition adjuvanttreatment is generallyindicated for tumors withgrowth on follow-upimaging even in theabsence of symptoms

bull The role of chemotherapyis in evolution

bull Chemotherapy may beimplemented for the treatment ofinoperable or partially resectedgliomas

bull The decision to use postoperativeadjuvant therapy also depends ona lesions perceived malignanttendencies as the potentialbenefits of radiation therapy andchemotherapy must be weighedagainst their morbidity

bull The decision to use postoperativeadjuvant therapy also depends ona lesions perceived malignanttendencies as the potentialbenefits of radiation therapy andchemotherapy must be weighedagainst their morbidity

10202016 24unusual giomas

Pleomorphic xanthoastrocytoma

bull First described by Kepsand colleuges in 1979

bull Represents lt1 of allastrocytomas

bull May be found alongentire neuraxis mostlysupratentorial( temporallobe)

bull Cerebellum and soinalcord are the other sites

bull Pts present in 2nd and 3rd

decadesbull Median age at 14 yearsbull Both sexes are affected

equallybull 70-80 pts present with

seizures and headachefocal neuro deficits

bull Rarely tumor mayhemmorrhage

10202016 25unusual giomas

bull Supratentorail are mostoften cortical and meningesbased

bull Chronic seizuresbull Grossely these are firm

tumors variable in colorrelatively avascular

bull Typically invade the piaarachnoid space

bull 13 pts have involvementof all the three meninges

bull Cyst fluid is xanthochromic

bull Very specific microscpoiccriteria for diagnosis are

1 Pleomorphic andxanthomatous astrocytes

2 Perivascular lymphocytes3 Non infilterating histology4 Reticulin stainingbull Pleomorphism can be as

vast as glioblastoma orsarcomatous tumors

10202016 26unusual giomas

bull IHC Staining revealsspindle cells and roundpleomorphic cellspositive for S-100 andGFAP

bull Staining forsynaptophysin and NSEmay be also present

bull Some workers havereported them to be asubtype of ganglioglioma

bull Association between theNeurofibromatosis Sturge Weber Syndrome

bull Loss of chr 9 and gain ofchromosome 7 have beenfound

10202016 27unusual giomas

Imaging

bull CTbull PXAs are typically hypo

or isodense and may bewell or poorlydemarcated usually withno or little surroundingoedema

bull Calcification is rarebull Due to its superficial

location it may causescalloping of theoverlying bone

10202016 28unusual giomas

bull MRIbull T1

ndash solid component iso tohypointense cf grey matter

ndash cystic component low signalndash leptomeningeal involvement

seen in over 70 of casesbull T1 C+ (Gd)

ndash solid component usuallyenhances vividly

bull T2ndash solid component iso to

hyperintense cf grey matterndash cystic component high signalndash on T2 FLAIR sequence cystic

areas show hyperintensityrelative to CSF due to higherprotein contents

ndash little surrounding vasogenicoedema

10202016 29unusual giomas

Management amp Outcome

bull Quite rare tumorsbull Surgical excision appears to

be best favoured modalitybull Actuarial survival trates are

91 at 5 years and 82 at10 years

bull Median survival 18 years

bull Total resection should beattempted in every case asfar as possible

bull Resection of cyst lining isnot indicated

bull Data regarding the use ofadjuvant therapy at thistime are limited to few casereports only

bull Pts who had incompleteresection high mitoticindex necrosis should bereserved for strict vigilanceand CEMRI on follow ups

10202016 30unusual giomas

bull At progression repeatresection be attempted

bull Proression should betaken as aggressivetumor type

bull Malignanttransformation maynecessitate adjuvanttherapy

10202016 31unusual giomas

bull Br J Neurosurg 2013 Dec27(6)759-64 doi 103109026886972013776666 Epub 2013 Mar 20

bull Pleomorphic xanthoastrocytoma long-term results of surgical treatment and analysis of prognosticfactors

bull Gallo P1 Cecchi PC Locatelli F Rizzo P Ghimenton C Gerosa M Pinna G

bull BACKGROUND

bull Pleomorphic Xanthoastrocytoma (PXA) is a rare brain tumour most commonly affecting children andyoung adults To date only few data regarding the long-term follow-up of these patients after surgery are

available The aim of this study is to describe our single-institution experience in the surgicalmanagement of this particular glioma over a period of over 18 years

bull METHODS

bull We performed a retrospective review of all cases of PXA (40 patients) operated upon at theDepartment of Neurosurgery of Verona Italy between 1990 and 2008 The impact of clinical radiologicalsurgical and histological factors on overall survival (OS) and progression-free survival (PFS) was analysed bymeans of univariate and multivariate models

bull FINDINGS

bull We achieved a gross total resection (GTR) in 65 of patients Histological diagnosis was of grade II in 80anaplastic features were present in the remaining 20 Adjuvant treatment radiotherapy or chemo-

radiotherapy was administered in 40 of the cases Median follow-up was 74 months OS at 5- and10 years was 7632 and 6824 respectively PFS at 5- and 10 years was 71 and 58respectively In the multivariate model histological grade extent of resection and age at diagnosis (le 30years vs gt 30 years) were the only independent prognostic factors for both OS and PFS

bull CONCLUSIONS

bull Our retrospective long-term study confirms the relatively favourable prognosis associatedwith PXA Young patients with a low-grade tumour (WHO grade II) who underwent GTR carrythe longest OS and PFS

10202016 32unusual giomas

bull Brain Tumor Pathol 2006 Apr23(1)55-63

bull A case of anaplastic pleomorphic xanthoastrocytoma presenting with tumor bleeding and cerebrospinal fluid dissemination

bull Asano K1 Miyamoto S Kubo O Kikkukawa T Yagihashi A Ohkuma H

bull Pleomorphic xanthoastrocytoma (PXA) has been considered an astrocytic tumorwith a relatively favorable prognosis However PXA cases having several recurrent patterns with poor prognosis have been reported in recent years and a new concept of anaplastic PXA has been proposed The present case was a 59-year-old woman who presented with tumor bleeding onset and cerebrospinal fluid dissemination The patient had sudden-onset right hemiparesis aphasia and consciousness disturbance and was admitted to a local area hospital After emergency surgery had removed the hematoma postoperative contrast-enhanced CT scan revealed a left temporal tumor A second surgery was therefore performed for initial tumor removal 2 months later Histopathological findings showed that the tumor was typical PXA with strong pleomorphism and xanthomatous changes and contained an ependymoma-like component in the center area However endothelial proliferation and mitosis were more remarkable compared to ordinary PXA The MIB-1 labeling index was 98 high From these findings the histopathological diagnosis was anaplastic PXA The patient underwent surgery to remove recurrent tumors 5 and 16 months later The patient died 36 months after the first onset and CT revealed glioblastoma-like

findings and cerebrospinal fluid dissemination This case report is the first case in which PXA presented with tumor bleeding onset Histopathological findings suggested anaplastic PXA from the first surgical specimens and PXA recurred many times We thus believe that the patient displayed primary anaplastic PXA rather than secondary anaplastic PXA that results in malignant transformation10202016 33unusual giomas

Ganglioglioma

bull WHO grade 1bull Initially described by

Perkins in 1926bull Benign well

differentiatedneuroepithelial tumors

bull Most commonly found intemporal lobe though canoccur anywhere in CNS

bull Commonly found inyoung patients withepilepsy

bull Most commonly notedtumor in temporal lobeepilepsy patients

bull Have been described inbrain steem spinal cordand cerebellum

bull Account for 1 ofprimary brain tumors

10202016 34unusual giomas

bull Male dominencebull Most common features

are seizures headachedizziness ataxiaprogressive weakness

bull Malignanttransformation is rare

bull Histology showsimmature mixture ofabnormal neural andglial elements

bull Macroscopically appearsolid or cystic

bull Cystic are welldelineatedfromsurrounding brain

bull Calcification is common

10202016 35unusual giomas

bull IHC staing shows reactivity for GFAP S-100 andvimentin

bull Neurons show synaptophysin class III betatubulin NFP chromogranin reactivity

10202016 36unusual giomas

Imaging

bull Highly variable imagingcharacters

bull No special typicalimaging criteria

bull Enhancement patternsare highly variable

bull Iso to hypointense onT1WI

bull Heterogenous andhyperintense on T2WI

bull Can contain solid cyticcalcified components

10202016 37unusual giomas

bull FDG PET scan showshypometabolism oreumetabolism in thesepatients

10202016 38unusual giomas

Management And Outcome

bull Gold standard is grosstotal resection

bull Seizure control aftersurgery is as high as80

bull Survival rates are highbull Event free survival of

low grade and highgrade gangiogioma are57 vs 15

bull The optimal surgicalresection depends uponthe eloquent areasinvolved in the vicinityof the tumor

bull The use ofchemotherapy is notwell established

bull Typically they shouldbe allowed for chemoand radiotherapy

10202016 39unusual giomas

bull The use of adjuvant therapy is warranted in thosecases which harbour residual lesions or features ofanaplasia

bull Potential harm to nearby brain areas preclude thejudicious use of radiation

10202016 40unusual giomas

bull Brain Tumor Res Treat 2014 Oct 2(2) 49ndash55

bull Treatment and Outcomes for Gangliogliomas A Single-Center Review of 16 Patients

bull Jye Young Song Jeong Hoon Kim Young Hyun Cho Chang Jin Kim and Eun Jung Lee

bull Ganglioglioma is a rare and slowly growing benign tumor We investigated the outcomes of patients who underwent different combination treatments

bull Methods

bull Between 1998 and 2012 16 patients including 11 men and 5 women with a median age of 125 years (range 25-65 years) were treated for intracranial gangliogliomas at our institution The median follow-up period was 57 years (range 48 days-156 years) Fifteen cases were included in the outcome assessment because one patient was lost to follow-up Complete resection was achieved in 8 (53) patients Six (40) patients underwent incomplete resection with or without adjuvant radiotherapy and one patient with a brainstem tumor underwent only stereotactic biopsy

bull Results

bull Gangliogliomas predominantly affected young (875) male patients and most frequently presented with seizures (64) Of eight patients who underwent complete resection seven did not show recurrence whereas only three of six with incomplete resection showed no recurrence Four patients with recurrence received salvage treatments (two repeat surgeries and two radiosurgeries) A tumor control rate of 93 (1415) was achieved at the last follow-up No recurrence or malignant changes were observed after a median follow-up of 12 and 45 years in four patients who received gamma knife (GK) radiosurgery as adjuvant and salvage treatment

bull Conclusion

bull Complete resection produced the best outcomes and incomplete resection followed by adjuvant or salvage treatments showed favorable outcomes In patients who are not eligible for complete resection because of tumor location or potential neurologic deficits following surgery GK radiosurgery should be considered for the treatment of residual or recurrent tumors

10202016 41unusual giomas

Papillary tumours of pineal

bull WHO grade II AND IIIbull Relatively new edition to WHObull They are equal in both sexesbull Reach medical attention due to headache mental

status changes and altered visionbull Contain eosinophilic cells with distinctive

borders with large pleomorphic nucleibull Positive for cytokeratin synaptophysin stain

variably for GFAP vimentin S-100

10202016 unusual giomas 42

Imaging

bull Generally found to be diffusely enhancinglesion of pineal region

bull Post 3rd ventricle with HCPbull Appear well demarcated from thalamus and

cerebellum

10202016 unusual giomas 43

Mangement

bull Standard treatment is excisionbull Grow slowly and donot transform to

malignant tumorsbull Local recurrence is however possiblebull Radiotherapy appears to provide good control

in local recurrence

10202016 unusual giomas 44

Choroid Glioma Of Third Ventricle

bull WHO Grade IIbull Ist recognised by WHO in

1998bull Arise in the anterior third

ventriclebull Commonly come to

attention after HCPbull Appera to contain in 3rd

ventricle and thought toarise from ventricularsurface

bull Composed of GFAP andvimentin positive cells inmucinous matrix

bull Nuclei are uniform insize and shape

bull Indolent growthbull May show EGF receptor

positivitybull Some suggets these tumor

arise from the lminaterminalis andventricular tanycytes

10202016 45unusual giomas

Imaging

bull Commonly fill 3rd

ventriclebull Diffusely enhance with

contrastbull Appear to infiltrate the

thalamusbull Large tumors may be

difficult to differentiatefrom suprasellar masses

10202016 46unusual giomas

10202016 47unusual giomas

bull Surgical management is limited by locationbull Access through the dialted ventricle and lamina

terminalis may be challangingbull Subtotal resection is thus commonbull Role of adjuvant radiotherapy is unknown

10202016 48unusual giomas

bull Acta Neurochir (Wien) 2008 Nov150(11)1183-91 doi 101007s00701-008-0014-6 Epub

2008 Oct 21

bull Chordoid glioma of the third ventricle

bull Vanhauwaert DJ1 Clement F Van Dorpe J Deruytter MJKGROUND

bull Chordoid glioma is a rare tumour (World Health Organisation grade II) originating from the third ventricle

with both glial and chordoid features It was first described by Brat in 1998 Since there is no detailed

information available on the outcome after surgery and adjuvant treatment we reviewed the literature

bull METHODS

bull A literature search through PUBMED revealed 50 cases of chordoid glioma Most reports

were found in pathology journals Information on the postoperative course was sometimes

very limited We reviewed the available literature and studied in detail the presenting

symptoms mortality and postoperative complications in relation to the extent of resective

surgery as well as the importance of adjuvant treatment

bull CONCLUSIONS

bull Mortality in the immediate postoperative period is 32 and is higher after gross total

resection as compared to subtotal resection Non-fatal postoperative complications are

hypothalamic disorders and mental alterations Gross total resection is the treatment of

choice since no recurrence has been reported after macroscopically complete resection

but this is often difficult because of the location and adherence to the hypothalamus The

role of postoperative radiotherapy is uncertain There is some indication that radiosurgery

with or without conventional irradiation is superior to conventional radiation alone Planned

subtotal resection followed by stereotactic radiosurgery can be a safe and effective

alternative in a patient in whom gross total resection is considered to be too risky

There is no report on the use of chemotherapy in the treatment of chordoid gliomas More

information about the optimal treatment strategy is needed and more reports are also needed10202016 49unusual giomas

bull Neurologist 2011 Jan17(1)52bull Chordoid glioma a case report and literature reviewbull Liu WP1 Cheng JX Yi XC Zhen HN Fei Z Li Q Zhang Xbull chordoid glioma is a rare tumor (World Health Organization grade II) with both

glial and chordoid features often located in the suprasellar region and anterior third ventricle It was first described by Brat in 1998 Because there is no detailed information available from the clinical perspective we reviewed the literature

bull METHODS

bull a literature search through PUBMED and CNKI revealed 64 cases of chordoid glioma Information on the clinical course was very limited We reviewed the literature and studied the pathologic and imaging features postoperative mortality and morbidity in relation to surgical extension and approaches and the importance of adjuvant treatment

bull CONCLUSIONS bull mortality in the immediate postoperative period is 28 and postoperative

morbidity is 60 which are statistically higher after gross total resection as compared with subtotal resection Translamina terminalis approach is considered to be the best approach The current study cannot document that patients have longer survival and higher quality of life after gross total resection than subtotal resection The role of postoperative radiotherapy is uncertain and there is no report on the use of chemotherapy More information about the optimal treatment strategy is needed

10202016 50unusual giomas

Conclusion

bull They are identified by their unique morphologicalfeatures

bull Many of these have indolent course and identified byepilepsy

bull Potentail diagnosis should be considered in youngpatients with seizures and cortically based lesions

bull Correct diagnosis requires experiencedneuropathologisrt

bull As more experienced is gained more knowledgetowards better understanding shall provide betteroutcomes in these patients

10202016 unusual giomas 51

10202016 52unusual giomas

Content supportndash youmann neurosurgery textbook

bull NO standard careprotocols have beendefined

bull As these are newlydefined and raretumors

bull Surgical resection isfavoured if locationfavours

bull Mean progression freesurvival is 25 months

bull Mean overall survival is60 months

bull Reduced survival isbecause of inability toachieve gross resection

10202016 23unusual giomas

bull The usual indications foradjuvant treatment(chemotherapy orradiation therapy)include tumor recurrenceafter initial completeresection or symptomatictumors

bull In addition adjuvanttreatment is generallyindicated for tumors withgrowth on follow-upimaging even in theabsence of symptoms

bull The role of chemotherapyis in evolution

bull Chemotherapy may beimplemented for the treatment ofinoperable or partially resectedgliomas

bull The decision to use postoperativeadjuvant therapy also depends ona lesions perceived malignanttendencies as the potentialbenefits of radiation therapy andchemotherapy must be weighedagainst their morbidity

bull The decision to use postoperativeadjuvant therapy also depends ona lesions perceived malignanttendencies as the potentialbenefits of radiation therapy andchemotherapy must be weighedagainst their morbidity

10202016 24unusual giomas

Pleomorphic xanthoastrocytoma

bull First described by Kepsand colleuges in 1979

bull Represents lt1 of allastrocytomas

bull May be found alongentire neuraxis mostlysupratentorial( temporallobe)

bull Cerebellum and soinalcord are the other sites

bull Pts present in 2nd and 3rd

decadesbull Median age at 14 yearsbull Both sexes are affected

equallybull 70-80 pts present with

seizures and headachefocal neuro deficits

bull Rarely tumor mayhemmorrhage

10202016 25unusual giomas

bull Supratentorail are mostoften cortical and meningesbased

bull Chronic seizuresbull Grossely these are firm

tumors variable in colorrelatively avascular

bull Typically invade the piaarachnoid space

bull 13 pts have involvementof all the three meninges

bull Cyst fluid is xanthochromic

bull Very specific microscpoiccriteria for diagnosis are

1 Pleomorphic andxanthomatous astrocytes

2 Perivascular lymphocytes3 Non infilterating histology4 Reticulin stainingbull Pleomorphism can be as

vast as glioblastoma orsarcomatous tumors

10202016 26unusual giomas

bull IHC Staining revealsspindle cells and roundpleomorphic cellspositive for S-100 andGFAP

bull Staining forsynaptophysin and NSEmay be also present

bull Some workers havereported them to be asubtype of ganglioglioma

bull Association between theNeurofibromatosis Sturge Weber Syndrome

bull Loss of chr 9 and gain ofchromosome 7 have beenfound

10202016 27unusual giomas

Imaging

bull CTbull PXAs are typically hypo

or isodense and may bewell or poorlydemarcated usually withno or little surroundingoedema

bull Calcification is rarebull Due to its superficial

location it may causescalloping of theoverlying bone

10202016 28unusual giomas

bull MRIbull T1

ndash solid component iso tohypointense cf grey matter

ndash cystic component low signalndash leptomeningeal involvement

seen in over 70 of casesbull T1 C+ (Gd)

ndash solid component usuallyenhances vividly

bull T2ndash solid component iso to

hyperintense cf grey matterndash cystic component high signalndash on T2 FLAIR sequence cystic

areas show hyperintensityrelative to CSF due to higherprotein contents

ndash little surrounding vasogenicoedema

10202016 29unusual giomas

Management amp Outcome

bull Quite rare tumorsbull Surgical excision appears to

be best favoured modalitybull Actuarial survival trates are

91 at 5 years and 82 at10 years

bull Median survival 18 years

bull Total resection should beattempted in every case asfar as possible

bull Resection of cyst lining isnot indicated

bull Data regarding the use ofadjuvant therapy at thistime are limited to few casereports only

bull Pts who had incompleteresection high mitoticindex necrosis should bereserved for strict vigilanceand CEMRI on follow ups

10202016 30unusual giomas

bull At progression repeatresection be attempted

bull Proression should betaken as aggressivetumor type

bull Malignanttransformation maynecessitate adjuvanttherapy

10202016 31unusual giomas

bull Br J Neurosurg 2013 Dec27(6)759-64 doi 103109026886972013776666 Epub 2013 Mar 20

bull Pleomorphic xanthoastrocytoma long-term results of surgical treatment and analysis of prognosticfactors

bull Gallo P1 Cecchi PC Locatelli F Rizzo P Ghimenton C Gerosa M Pinna G

bull BACKGROUND

bull Pleomorphic Xanthoastrocytoma (PXA) is a rare brain tumour most commonly affecting children andyoung adults To date only few data regarding the long-term follow-up of these patients after surgery are

available The aim of this study is to describe our single-institution experience in the surgicalmanagement of this particular glioma over a period of over 18 years

bull METHODS

bull We performed a retrospective review of all cases of PXA (40 patients) operated upon at theDepartment of Neurosurgery of Verona Italy between 1990 and 2008 The impact of clinical radiologicalsurgical and histological factors on overall survival (OS) and progression-free survival (PFS) was analysed bymeans of univariate and multivariate models

bull FINDINGS

bull We achieved a gross total resection (GTR) in 65 of patients Histological diagnosis was of grade II in 80anaplastic features were present in the remaining 20 Adjuvant treatment radiotherapy or chemo-

radiotherapy was administered in 40 of the cases Median follow-up was 74 months OS at 5- and10 years was 7632 and 6824 respectively PFS at 5- and 10 years was 71 and 58respectively In the multivariate model histological grade extent of resection and age at diagnosis (le 30years vs gt 30 years) were the only independent prognostic factors for both OS and PFS

bull CONCLUSIONS

bull Our retrospective long-term study confirms the relatively favourable prognosis associatedwith PXA Young patients with a low-grade tumour (WHO grade II) who underwent GTR carrythe longest OS and PFS

10202016 32unusual giomas

bull Brain Tumor Pathol 2006 Apr23(1)55-63

bull A case of anaplastic pleomorphic xanthoastrocytoma presenting with tumor bleeding and cerebrospinal fluid dissemination

bull Asano K1 Miyamoto S Kubo O Kikkukawa T Yagihashi A Ohkuma H

bull Pleomorphic xanthoastrocytoma (PXA) has been considered an astrocytic tumorwith a relatively favorable prognosis However PXA cases having several recurrent patterns with poor prognosis have been reported in recent years and a new concept of anaplastic PXA has been proposed The present case was a 59-year-old woman who presented with tumor bleeding onset and cerebrospinal fluid dissemination The patient had sudden-onset right hemiparesis aphasia and consciousness disturbance and was admitted to a local area hospital After emergency surgery had removed the hematoma postoperative contrast-enhanced CT scan revealed a left temporal tumor A second surgery was therefore performed for initial tumor removal 2 months later Histopathological findings showed that the tumor was typical PXA with strong pleomorphism and xanthomatous changes and contained an ependymoma-like component in the center area However endothelial proliferation and mitosis were more remarkable compared to ordinary PXA The MIB-1 labeling index was 98 high From these findings the histopathological diagnosis was anaplastic PXA The patient underwent surgery to remove recurrent tumors 5 and 16 months later The patient died 36 months after the first onset and CT revealed glioblastoma-like

findings and cerebrospinal fluid dissemination This case report is the first case in which PXA presented with tumor bleeding onset Histopathological findings suggested anaplastic PXA from the first surgical specimens and PXA recurred many times We thus believe that the patient displayed primary anaplastic PXA rather than secondary anaplastic PXA that results in malignant transformation10202016 33unusual giomas

Ganglioglioma

bull WHO grade 1bull Initially described by

Perkins in 1926bull Benign well

differentiatedneuroepithelial tumors

bull Most commonly found intemporal lobe though canoccur anywhere in CNS

bull Commonly found inyoung patients withepilepsy

bull Most commonly notedtumor in temporal lobeepilepsy patients

bull Have been described inbrain steem spinal cordand cerebellum

bull Account for 1 ofprimary brain tumors

10202016 34unusual giomas

bull Male dominencebull Most common features

are seizures headachedizziness ataxiaprogressive weakness

bull Malignanttransformation is rare

bull Histology showsimmature mixture ofabnormal neural andglial elements

bull Macroscopically appearsolid or cystic

bull Cystic are welldelineatedfromsurrounding brain

bull Calcification is common

10202016 35unusual giomas

bull IHC staing shows reactivity for GFAP S-100 andvimentin

bull Neurons show synaptophysin class III betatubulin NFP chromogranin reactivity

10202016 36unusual giomas

Imaging

bull Highly variable imagingcharacters

bull No special typicalimaging criteria

bull Enhancement patternsare highly variable

bull Iso to hypointense onT1WI

bull Heterogenous andhyperintense on T2WI

bull Can contain solid cyticcalcified components

10202016 37unusual giomas

bull FDG PET scan showshypometabolism oreumetabolism in thesepatients

10202016 38unusual giomas

Management And Outcome

bull Gold standard is grosstotal resection

bull Seizure control aftersurgery is as high as80

bull Survival rates are highbull Event free survival of

low grade and highgrade gangiogioma are57 vs 15

bull The optimal surgicalresection depends uponthe eloquent areasinvolved in the vicinityof the tumor

bull The use ofchemotherapy is notwell established

bull Typically they shouldbe allowed for chemoand radiotherapy

10202016 39unusual giomas

bull The use of adjuvant therapy is warranted in thosecases which harbour residual lesions or features ofanaplasia

bull Potential harm to nearby brain areas preclude thejudicious use of radiation

10202016 40unusual giomas

bull Brain Tumor Res Treat 2014 Oct 2(2) 49ndash55

bull Treatment and Outcomes for Gangliogliomas A Single-Center Review of 16 Patients

bull Jye Young Song Jeong Hoon Kim Young Hyun Cho Chang Jin Kim and Eun Jung Lee

bull Ganglioglioma is a rare and slowly growing benign tumor We investigated the outcomes of patients who underwent different combination treatments

bull Methods

bull Between 1998 and 2012 16 patients including 11 men and 5 women with a median age of 125 years (range 25-65 years) were treated for intracranial gangliogliomas at our institution The median follow-up period was 57 years (range 48 days-156 years) Fifteen cases were included in the outcome assessment because one patient was lost to follow-up Complete resection was achieved in 8 (53) patients Six (40) patients underwent incomplete resection with or without adjuvant radiotherapy and one patient with a brainstem tumor underwent only stereotactic biopsy

bull Results

bull Gangliogliomas predominantly affected young (875) male patients and most frequently presented with seizures (64) Of eight patients who underwent complete resection seven did not show recurrence whereas only three of six with incomplete resection showed no recurrence Four patients with recurrence received salvage treatments (two repeat surgeries and two radiosurgeries) A tumor control rate of 93 (1415) was achieved at the last follow-up No recurrence or malignant changes were observed after a median follow-up of 12 and 45 years in four patients who received gamma knife (GK) radiosurgery as adjuvant and salvage treatment

bull Conclusion

bull Complete resection produced the best outcomes and incomplete resection followed by adjuvant or salvage treatments showed favorable outcomes In patients who are not eligible for complete resection because of tumor location or potential neurologic deficits following surgery GK radiosurgery should be considered for the treatment of residual or recurrent tumors

10202016 41unusual giomas

Papillary tumours of pineal

bull WHO grade II AND IIIbull Relatively new edition to WHObull They are equal in both sexesbull Reach medical attention due to headache mental

status changes and altered visionbull Contain eosinophilic cells with distinctive

borders with large pleomorphic nucleibull Positive for cytokeratin synaptophysin stain

variably for GFAP vimentin S-100

10202016 unusual giomas 42

Imaging

bull Generally found to be diffusely enhancinglesion of pineal region

bull Post 3rd ventricle with HCPbull Appear well demarcated from thalamus and

cerebellum

10202016 unusual giomas 43

Mangement

bull Standard treatment is excisionbull Grow slowly and donot transform to

malignant tumorsbull Local recurrence is however possiblebull Radiotherapy appears to provide good control

in local recurrence

10202016 unusual giomas 44

Choroid Glioma Of Third Ventricle

bull WHO Grade IIbull Ist recognised by WHO in

1998bull Arise in the anterior third

ventriclebull Commonly come to

attention after HCPbull Appera to contain in 3rd

ventricle and thought toarise from ventricularsurface

bull Composed of GFAP andvimentin positive cells inmucinous matrix

bull Nuclei are uniform insize and shape

bull Indolent growthbull May show EGF receptor

positivitybull Some suggets these tumor

arise from the lminaterminalis andventricular tanycytes

10202016 45unusual giomas

Imaging

bull Commonly fill 3rd

ventriclebull Diffusely enhance with

contrastbull Appear to infiltrate the

thalamusbull Large tumors may be

difficult to differentiatefrom suprasellar masses

10202016 46unusual giomas

10202016 47unusual giomas

bull Surgical management is limited by locationbull Access through the dialted ventricle and lamina

terminalis may be challangingbull Subtotal resection is thus commonbull Role of adjuvant radiotherapy is unknown

10202016 48unusual giomas

bull Acta Neurochir (Wien) 2008 Nov150(11)1183-91 doi 101007s00701-008-0014-6 Epub

2008 Oct 21

bull Chordoid glioma of the third ventricle

bull Vanhauwaert DJ1 Clement F Van Dorpe J Deruytter MJKGROUND

bull Chordoid glioma is a rare tumour (World Health Organisation grade II) originating from the third ventricle

with both glial and chordoid features It was first described by Brat in 1998 Since there is no detailed

information available on the outcome after surgery and adjuvant treatment we reviewed the literature

bull METHODS

bull A literature search through PUBMED revealed 50 cases of chordoid glioma Most reports

were found in pathology journals Information on the postoperative course was sometimes

very limited We reviewed the available literature and studied in detail the presenting

symptoms mortality and postoperative complications in relation to the extent of resective

surgery as well as the importance of adjuvant treatment

bull CONCLUSIONS

bull Mortality in the immediate postoperative period is 32 and is higher after gross total

resection as compared to subtotal resection Non-fatal postoperative complications are

hypothalamic disorders and mental alterations Gross total resection is the treatment of

choice since no recurrence has been reported after macroscopically complete resection

but this is often difficult because of the location and adherence to the hypothalamus The

role of postoperative radiotherapy is uncertain There is some indication that radiosurgery

with or without conventional irradiation is superior to conventional radiation alone Planned

subtotal resection followed by stereotactic radiosurgery can be a safe and effective

alternative in a patient in whom gross total resection is considered to be too risky

There is no report on the use of chemotherapy in the treatment of chordoid gliomas More

information about the optimal treatment strategy is needed and more reports are also needed10202016 49unusual giomas

bull Neurologist 2011 Jan17(1)52bull Chordoid glioma a case report and literature reviewbull Liu WP1 Cheng JX Yi XC Zhen HN Fei Z Li Q Zhang Xbull chordoid glioma is a rare tumor (World Health Organization grade II) with both

glial and chordoid features often located in the suprasellar region and anterior third ventricle It was first described by Brat in 1998 Because there is no detailed information available from the clinical perspective we reviewed the literature

bull METHODS

bull a literature search through PUBMED and CNKI revealed 64 cases of chordoid glioma Information on the clinical course was very limited We reviewed the literature and studied the pathologic and imaging features postoperative mortality and morbidity in relation to surgical extension and approaches and the importance of adjuvant treatment

bull CONCLUSIONS bull mortality in the immediate postoperative period is 28 and postoperative

morbidity is 60 which are statistically higher after gross total resection as compared with subtotal resection Translamina terminalis approach is considered to be the best approach The current study cannot document that patients have longer survival and higher quality of life after gross total resection than subtotal resection The role of postoperative radiotherapy is uncertain and there is no report on the use of chemotherapy More information about the optimal treatment strategy is needed

10202016 50unusual giomas

Conclusion

bull They are identified by their unique morphologicalfeatures

bull Many of these have indolent course and identified byepilepsy

bull Potentail diagnosis should be considered in youngpatients with seizures and cortically based lesions

bull Correct diagnosis requires experiencedneuropathologisrt

bull As more experienced is gained more knowledgetowards better understanding shall provide betteroutcomes in these patients

10202016 unusual giomas 51

10202016 52unusual giomas

Content supportndash youmann neurosurgery textbook

bull The usual indications foradjuvant treatment(chemotherapy orradiation therapy)include tumor recurrenceafter initial completeresection or symptomatictumors

bull In addition adjuvanttreatment is generallyindicated for tumors withgrowth on follow-upimaging even in theabsence of symptoms

bull The role of chemotherapyis in evolution

bull Chemotherapy may beimplemented for the treatment ofinoperable or partially resectedgliomas

bull The decision to use postoperativeadjuvant therapy also depends ona lesions perceived malignanttendencies as the potentialbenefits of radiation therapy andchemotherapy must be weighedagainst their morbidity

bull The decision to use postoperativeadjuvant therapy also depends ona lesions perceived malignanttendencies as the potentialbenefits of radiation therapy andchemotherapy must be weighedagainst their morbidity

10202016 24unusual giomas

Pleomorphic xanthoastrocytoma

bull First described by Kepsand colleuges in 1979

bull Represents lt1 of allastrocytomas

bull May be found alongentire neuraxis mostlysupratentorial( temporallobe)

bull Cerebellum and soinalcord are the other sites

bull Pts present in 2nd and 3rd

decadesbull Median age at 14 yearsbull Both sexes are affected

equallybull 70-80 pts present with

seizures and headachefocal neuro deficits

bull Rarely tumor mayhemmorrhage

10202016 25unusual giomas

bull Supratentorail are mostoften cortical and meningesbased

bull Chronic seizuresbull Grossely these are firm

tumors variable in colorrelatively avascular

bull Typically invade the piaarachnoid space

bull 13 pts have involvementof all the three meninges

bull Cyst fluid is xanthochromic

bull Very specific microscpoiccriteria for diagnosis are

1 Pleomorphic andxanthomatous astrocytes

2 Perivascular lymphocytes3 Non infilterating histology4 Reticulin stainingbull Pleomorphism can be as

vast as glioblastoma orsarcomatous tumors

10202016 26unusual giomas

bull IHC Staining revealsspindle cells and roundpleomorphic cellspositive for S-100 andGFAP

bull Staining forsynaptophysin and NSEmay be also present

bull Some workers havereported them to be asubtype of ganglioglioma

bull Association between theNeurofibromatosis Sturge Weber Syndrome

bull Loss of chr 9 and gain ofchromosome 7 have beenfound

10202016 27unusual giomas

Imaging

bull CTbull PXAs are typically hypo

or isodense and may bewell or poorlydemarcated usually withno or little surroundingoedema

bull Calcification is rarebull Due to its superficial

location it may causescalloping of theoverlying bone

10202016 28unusual giomas

bull MRIbull T1

ndash solid component iso tohypointense cf grey matter

ndash cystic component low signalndash leptomeningeal involvement

seen in over 70 of casesbull T1 C+ (Gd)

ndash solid component usuallyenhances vividly

bull T2ndash solid component iso to

hyperintense cf grey matterndash cystic component high signalndash on T2 FLAIR sequence cystic

areas show hyperintensityrelative to CSF due to higherprotein contents

ndash little surrounding vasogenicoedema

10202016 29unusual giomas

Management amp Outcome

bull Quite rare tumorsbull Surgical excision appears to

be best favoured modalitybull Actuarial survival trates are

91 at 5 years and 82 at10 years

bull Median survival 18 years

bull Total resection should beattempted in every case asfar as possible

bull Resection of cyst lining isnot indicated

bull Data regarding the use ofadjuvant therapy at thistime are limited to few casereports only

bull Pts who had incompleteresection high mitoticindex necrosis should bereserved for strict vigilanceand CEMRI on follow ups

10202016 30unusual giomas

bull At progression repeatresection be attempted

bull Proression should betaken as aggressivetumor type

bull Malignanttransformation maynecessitate adjuvanttherapy

10202016 31unusual giomas

bull Br J Neurosurg 2013 Dec27(6)759-64 doi 103109026886972013776666 Epub 2013 Mar 20

bull Pleomorphic xanthoastrocytoma long-term results of surgical treatment and analysis of prognosticfactors

bull Gallo P1 Cecchi PC Locatelli F Rizzo P Ghimenton C Gerosa M Pinna G

bull BACKGROUND

bull Pleomorphic Xanthoastrocytoma (PXA) is a rare brain tumour most commonly affecting children andyoung adults To date only few data regarding the long-term follow-up of these patients after surgery are

available The aim of this study is to describe our single-institution experience in the surgicalmanagement of this particular glioma over a period of over 18 years

bull METHODS

bull We performed a retrospective review of all cases of PXA (40 patients) operated upon at theDepartment of Neurosurgery of Verona Italy between 1990 and 2008 The impact of clinical radiologicalsurgical and histological factors on overall survival (OS) and progression-free survival (PFS) was analysed bymeans of univariate and multivariate models

bull FINDINGS

bull We achieved a gross total resection (GTR) in 65 of patients Histological diagnosis was of grade II in 80anaplastic features were present in the remaining 20 Adjuvant treatment radiotherapy or chemo-

radiotherapy was administered in 40 of the cases Median follow-up was 74 months OS at 5- and10 years was 7632 and 6824 respectively PFS at 5- and 10 years was 71 and 58respectively In the multivariate model histological grade extent of resection and age at diagnosis (le 30years vs gt 30 years) were the only independent prognostic factors for both OS and PFS

bull CONCLUSIONS

bull Our retrospective long-term study confirms the relatively favourable prognosis associatedwith PXA Young patients with a low-grade tumour (WHO grade II) who underwent GTR carrythe longest OS and PFS

10202016 32unusual giomas

bull Brain Tumor Pathol 2006 Apr23(1)55-63

bull A case of anaplastic pleomorphic xanthoastrocytoma presenting with tumor bleeding and cerebrospinal fluid dissemination

bull Asano K1 Miyamoto S Kubo O Kikkukawa T Yagihashi A Ohkuma H

bull Pleomorphic xanthoastrocytoma (PXA) has been considered an astrocytic tumorwith a relatively favorable prognosis However PXA cases having several recurrent patterns with poor prognosis have been reported in recent years and a new concept of anaplastic PXA has been proposed The present case was a 59-year-old woman who presented with tumor bleeding onset and cerebrospinal fluid dissemination The patient had sudden-onset right hemiparesis aphasia and consciousness disturbance and was admitted to a local area hospital After emergency surgery had removed the hematoma postoperative contrast-enhanced CT scan revealed a left temporal tumor A second surgery was therefore performed for initial tumor removal 2 months later Histopathological findings showed that the tumor was typical PXA with strong pleomorphism and xanthomatous changes and contained an ependymoma-like component in the center area However endothelial proliferation and mitosis were more remarkable compared to ordinary PXA The MIB-1 labeling index was 98 high From these findings the histopathological diagnosis was anaplastic PXA The patient underwent surgery to remove recurrent tumors 5 and 16 months later The patient died 36 months after the first onset and CT revealed glioblastoma-like

findings and cerebrospinal fluid dissemination This case report is the first case in which PXA presented with tumor bleeding onset Histopathological findings suggested anaplastic PXA from the first surgical specimens and PXA recurred many times We thus believe that the patient displayed primary anaplastic PXA rather than secondary anaplastic PXA that results in malignant transformation10202016 33unusual giomas

Ganglioglioma

bull WHO grade 1bull Initially described by

Perkins in 1926bull Benign well

differentiatedneuroepithelial tumors

bull Most commonly found intemporal lobe though canoccur anywhere in CNS

bull Commonly found inyoung patients withepilepsy

bull Most commonly notedtumor in temporal lobeepilepsy patients

bull Have been described inbrain steem spinal cordand cerebellum

bull Account for 1 ofprimary brain tumors

10202016 34unusual giomas

bull Male dominencebull Most common features

are seizures headachedizziness ataxiaprogressive weakness

bull Malignanttransformation is rare

bull Histology showsimmature mixture ofabnormal neural andglial elements

bull Macroscopically appearsolid or cystic

bull Cystic are welldelineatedfromsurrounding brain

bull Calcification is common

10202016 35unusual giomas

bull IHC staing shows reactivity for GFAP S-100 andvimentin

bull Neurons show synaptophysin class III betatubulin NFP chromogranin reactivity

10202016 36unusual giomas

Imaging

bull Highly variable imagingcharacters

bull No special typicalimaging criteria

bull Enhancement patternsare highly variable

bull Iso to hypointense onT1WI

bull Heterogenous andhyperintense on T2WI

bull Can contain solid cyticcalcified components

10202016 37unusual giomas

bull FDG PET scan showshypometabolism oreumetabolism in thesepatients

10202016 38unusual giomas

Management And Outcome

bull Gold standard is grosstotal resection

bull Seizure control aftersurgery is as high as80

bull Survival rates are highbull Event free survival of

low grade and highgrade gangiogioma are57 vs 15

bull The optimal surgicalresection depends uponthe eloquent areasinvolved in the vicinityof the tumor

bull The use ofchemotherapy is notwell established

bull Typically they shouldbe allowed for chemoand radiotherapy

10202016 39unusual giomas

bull The use of adjuvant therapy is warranted in thosecases which harbour residual lesions or features ofanaplasia

bull Potential harm to nearby brain areas preclude thejudicious use of radiation

10202016 40unusual giomas

bull Brain Tumor Res Treat 2014 Oct 2(2) 49ndash55

bull Treatment and Outcomes for Gangliogliomas A Single-Center Review of 16 Patients

bull Jye Young Song Jeong Hoon Kim Young Hyun Cho Chang Jin Kim and Eun Jung Lee

bull Ganglioglioma is a rare and slowly growing benign tumor We investigated the outcomes of patients who underwent different combination treatments

bull Methods

bull Between 1998 and 2012 16 patients including 11 men and 5 women with a median age of 125 years (range 25-65 years) were treated for intracranial gangliogliomas at our institution The median follow-up period was 57 years (range 48 days-156 years) Fifteen cases were included in the outcome assessment because one patient was lost to follow-up Complete resection was achieved in 8 (53) patients Six (40) patients underwent incomplete resection with or without adjuvant radiotherapy and one patient with a brainstem tumor underwent only stereotactic biopsy

bull Results

bull Gangliogliomas predominantly affected young (875) male patients and most frequently presented with seizures (64) Of eight patients who underwent complete resection seven did not show recurrence whereas only three of six with incomplete resection showed no recurrence Four patients with recurrence received salvage treatments (two repeat surgeries and two radiosurgeries) A tumor control rate of 93 (1415) was achieved at the last follow-up No recurrence or malignant changes were observed after a median follow-up of 12 and 45 years in four patients who received gamma knife (GK) radiosurgery as adjuvant and salvage treatment

bull Conclusion

bull Complete resection produced the best outcomes and incomplete resection followed by adjuvant or salvage treatments showed favorable outcomes In patients who are not eligible for complete resection because of tumor location or potential neurologic deficits following surgery GK radiosurgery should be considered for the treatment of residual or recurrent tumors

10202016 41unusual giomas

Papillary tumours of pineal

bull WHO grade II AND IIIbull Relatively new edition to WHObull They are equal in both sexesbull Reach medical attention due to headache mental

status changes and altered visionbull Contain eosinophilic cells with distinctive

borders with large pleomorphic nucleibull Positive for cytokeratin synaptophysin stain

variably for GFAP vimentin S-100

10202016 unusual giomas 42

Imaging

bull Generally found to be diffusely enhancinglesion of pineal region

bull Post 3rd ventricle with HCPbull Appear well demarcated from thalamus and

cerebellum

10202016 unusual giomas 43

Mangement

bull Standard treatment is excisionbull Grow slowly and donot transform to

malignant tumorsbull Local recurrence is however possiblebull Radiotherapy appears to provide good control

in local recurrence

10202016 unusual giomas 44

Choroid Glioma Of Third Ventricle

bull WHO Grade IIbull Ist recognised by WHO in

1998bull Arise in the anterior third

ventriclebull Commonly come to

attention after HCPbull Appera to contain in 3rd

ventricle and thought toarise from ventricularsurface

bull Composed of GFAP andvimentin positive cells inmucinous matrix

bull Nuclei are uniform insize and shape

bull Indolent growthbull May show EGF receptor

positivitybull Some suggets these tumor

arise from the lminaterminalis andventricular tanycytes

10202016 45unusual giomas

Imaging

bull Commonly fill 3rd

ventriclebull Diffusely enhance with

contrastbull Appear to infiltrate the

thalamusbull Large tumors may be

difficult to differentiatefrom suprasellar masses

10202016 46unusual giomas

10202016 47unusual giomas

bull Surgical management is limited by locationbull Access through the dialted ventricle and lamina

terminalis may be challangingbull Subtotal resection is thus commonbull Role of adjuvant radiotherapy is unknown

10202016 48unusual giomas

bull Acta Neurochir (Wien) 2008 Nov150(11)1183-91 doi 101007s00701-008-0014-6 Epub

2008 Oct 21

bull Chordoid glioma of the third ventricle

bull Vanhauwaert DJ1 Clement F Van Dorpe J Deruytter MJKGROUND

bull Chordoid glioma is a rare tumour (World Health Organisation grade II) originating from the third ventricle

with both glial and chordoid features It was first described by Brat in 1998 Since there is no detailed

information available on the outcome after surgery and adjuvant treatment we reviewed the literature

bull METHODS

bull A literature search through PUBMED revealed 50 cases of chordoid glioma Most reports

were found in pathology journals Information on the postoperative course was sometimes

very limited We reviewed the available literature and studied in detail the presenting

symptoms mortality and postoperative complications in relation to the extent of resective

surgery as well as the importance of adjuvant treatment

bull CONCLUSIONS

bull Mortality in the immediate postoperative period is 32 and is higher after gross total

resection as compared to subtotal resection Non-fatal postoperative complications are

hypothalamic disorders and mental alterations Gross total resection is the treatment of

choice since no recurrence has been reported after macroscopically complete resection

but this is often difficult because of the location and adherence to the hypothalamus The

role of postoperative radiotherapy is uncertain There is some indication that radiosurgery

with or without conventional irradiation is superior to conventional radiation alone Planned

subtotal resection followed by stereotactic radiosurgery can be a safe and effective

alternative in a patient in whom gross total resection is considered to be too risky

There is no report on the use of chemotherapy in the treatment of chordoid gliomas More

information about the optimal treatment strategy is needed and more reports are also needed10202016 49unusual giomas

bull Neurologist 2011 Jan17(1)52bull Chordoid glioma a case report and literature reviewbull Liu WP1 Cheng JX Yi XC Zhen HN Fei Z Li Q Zhang Xbull chordoid glioma is a rare tumor (World Health Organization grade II) with both

glial and chordoid features often located in the suprasellar region and anterior third ventricle It was first described by Brat in 1998 Because there is no detailed information available from the clinical perspective we reviewed the literature

bull METHODS

bull a literature search through PUBMED and CNKI revealed 64 cases of chordoid glioma Information on the clinical course was very limited We reviewed the literature and studied the pathologic and imaging features postoperative mortality and morbidity in relation to surgical extension and approaches and the importance of adjuvant treatment

bull CONCLUSIONS bull mortality in the immediate postoperative period is 28 and postoperative

morbidity is 60 which are statistically higher after gross total resection as compared with subtotal resection Translamina terminalis approach is considered to be the best approach The current study cannot document that patients have longer survival and higher quality of life after gross total resection than subtotal resection The role of postoperative radiotherapy is uncertain and there is no report on the use of chemotherapy More information about the optimal treatment strategy is needed

10202016 50unusual giomas

Conclusion

bull They are identified by their unique morphologicalfeatures

bull Many of these have indolent course and identified byepilepsy

bull Potentail diagnosis should be considered in youngpatients with seizures and cortically based lesions

bull Correct diagnosis requires experiencedneuropathologisrt

bull As more experienced is gained more knowledgetowards better understanding shall provide betteroutcomes in these patients

10202016 unusual giomas 51

10202016 52unusual giomas

Content supportndash youmann neurosurgery textbook

Pleomorphic xanthoastrocytoma

bull First described by Kepsand colleuges in 1979

bull Represents lt1 of allastrocytomas

bull May be found alongentire neuraxis mostlysupratentorial( temporallobe)

bull Cerebellum and soinalcord are the other sites

bull Pts present in 2nd and 3rd

decadesbull Median age at 14 yearsbull Both sexes are affected

equallybull 70-80 pts present with

seizures and headachefocal neuro deficits

bull Rarely tumor mayhemmorrhage

10202016 25unusual giomas

bull Supratentorail are mostoften cortical and meningesbased

bull Chronic seizuresbull Grossely these are firm

tumors variable in colorrelatively avascular

bull Typically invade the piaarachnoid space

bull 13 pts have involvementof all the three meninges

bull Cyst fluid is xanthochromic

bull Very specific microscpoiccriteria for diagnosis are

1 Pleomorphic andxanthomatous astrocytes

2 Perivascular lymphocytes3 Non infilterating histology4 Reticulin stainingbull Pleomorphism can be as

vast as glioblastoma orsarcomatous tumors

10202016 26unusual giomas

bull IHC Staining revealsspindle cells and roundpleomorphic cellspositive for S-100 andGFAP

bull Staining forsynaptophysin and NSEmay be also present

bull Some workers havereported them to be asubtype of ganglioglioma

bull Association between theNeurofibromatosis Sturge Weber Syndrome

bull Loss of chr 9 and gain ofchromosome 7 have beenfound

10202016 27unusual giomas

Imaging

bull CTbull PXAs are typically hypo

or isodense and may bewell or poorlydemarcated usually withno or little surroundingoedema

bull Calcification is rarebull Due to its superficial

location it may causescalloping of theoverlying bone

10202016 28unusual giomas

bull MRIbull T1

ndash solid component iso tohypointense cf grey matter

ndash cystic component low signalndash leptomeningeal involvement

seen in over 70 of casesbull T1 C+ (Gd)

ndash solid component usuallyenhances vividly

bull T2ndash solid component iso to

hyperintense cf grey matterndash cystic component high signalndash on T2 FLAIR sequence cystic

areas show hyperintensityrelative to CSF due to higherprotein contents

ndash little surrounding vasogenicoedema

10202016 29unusual giomas

Management amp Outcome

bull Quite rare tumorsbull Surgical excision appears to

be best favoured modalitybull Actuarial survival trates are

91 at 5 years and 82 at10 years

bull Median survival 18 years

bull Total resection should beattempted in every case asfar as possible

bull Resection of cyst lining isnot indicated

bull Data regarding the use ofadjuvant therapy at thistime are limited to few casereports only

bull Pts who had incompleteresection high mitoticindex necrosis should bereserved for strict vigilanceand CEMRI on follow ups

10202016 30unusual giomas

bull At progression repeatresection be attempted

bull Proression should betaken as aggressivetumor type

bull Malignanttransformation maynecessitate adjuvanttherapy

10202016 31unusual giomas

bull Br J Neurosurg 2013 Dec27(6)759-64 doi 103109026886972013776666 Epub 2013 Mar 20

bull Pleomorphic xanthoastrocytoma long-term results of surgical treatment and analysis of prognosticfactors

bull Gallo P1 Cecchi PC Locatelli F Rizzo P Ghimenton C Gerosa M Pinna G

bull BACKGROUND

bull Pleomorphic Xanthoastrocytoma (PXA) is a rare brain tumour most commonly affecting children andyoung adults To date only few data regarding the long-term follow-up of these patients after surgery are

available The aim of this study is to describe our single-institution experience in the surgicalmanagement of this particular glioma over a period of over 18 years

bull METHODS

bull We performed a retrospective review of all cases of PXA (40 patients) operated upon at theDepartment of Neurosurgery of Verona Italy between 1990 and 2008 The impact of clinical radiologicalsurgical and histological factors on overall survival (OS) and progression-free survival (PFS) was analysed bymeans of univariate and multivariate models

bull FINDINGS

bull We achieved a gross total resection (GTR) in 65 of patients Histological diagnosis was of grade II in 80anaplastic features were present in the remaining 20 Adjuvant treatment radiotherapy or chemo-

radiotherapy was administered in 40 of the cases Median follow-up was 74 months OS at 5- and10 years was 7632 and 6824 respectively PFS at 5- and 10 years was 71 and 58respectively In the multivariate model histological grade extent of resection and age at diagnosis (le 30years vs gt 30 years) were the only independent prognostic factors for both OS and PFS

bull CONCLUSIONS

bull Our retrospective long-term study confirms the relatively favourable prognosis associatedwith PXA Young patients with a low-grade tumour (WHO grade II) who underwent GTR carrythe longest OS and PFS

10202016 32unusual giomas

bull Brain Tumor Pathol 2006 Apr23(1)55-63

bull A case of anaplastic pleomorphic xanthoastrocytoma presenting with tumor bleeding and cerebrospinal fluid dissemination

bull Asano K1 Miyamoto S Kubo O Kikkukawa T Yagihashi A Ohkuma H

bull Pleomorphic xanthoastrocytoma (PXA) has been considered an astrocytic tumorwith a relatively favorable prognosis However PXA cases having several recurrent patterns with poor prognosis have been reported in recent years and a new concept of anaplastic PXA has been proposed The present case was a 59-year-old woman who presented with tumor bleeding onset and cerebrospinal fluid dissemination The patient had sudden-onset right hemiparesis aphasia and consciousness disturbance and was admitted to a local area hospital After emergency surgery had removed the hematoma postoperative contrast-enhanced CT scan revealed a left temporal tumor A second surgery was therefore performed for initial tumor removal 2 months later Histopathological findings showed that the tumor was typical PXA with strong pleomorphism and xanthomatous changes and contained an ependymoma-like component in the center area However endothelial proliferation and mitosis were more remarkable compared to ordinary PXA The MIB-1 labeling index was 98 high From these findings the histopathological diagnosis was anaplastic PXA The patient underwent surgery to remove recurrent tumors 5 and 16 months later The patient died 36 months after the first onset and CT revealed glioblastoma-like

findings and cerebrospinal fluid dissemination This case report is the first case in which PXA presented with tumor bleeding onset Histopathological findings suggested anaplastic PXA from the first surgical specimens and PXA recurred many times We thus believe that the patient displayed primary anaplastic PXA rather than secondary anaplastic PXA that results in malignant transformation10202016 33unusual giomas

Ganglioglioma

bull WHO grade 1bull Initially described by

Perkins in 1926bull Benign well

differentiatedneuroepithelial tumors

bull Most commonly found intemporal lobe though canoccur anywhere in CNS

bull Commonly found inyoung patients withepilepsy

bull Most commonly notedtumor in temporal lobeepilepsy patients

bull Have been described inbrain steem spinal cordand cerebellum

bull Account for 1 ofprimary brain tumors

10202016 34unusual giomas

bull Male dominencebull Most common features

are seizures headachedizziness ataxiaprogressive weakness

bull Malignanttransformation is rare

bull Histology showsimmature mixture ofabnormal neural andglial elements

bull Macroscopically appearsolid or cystic

bull Cystic are welldelineatedfromsurrounding brain

bull Calcification is common

10202016 35unusual giomas

bull IHC staing shows reactivity for GFAP S-100 andvimentin

bull Neurons show synaptophysin class III betatubulin NFP chromogranin reactivity

10202016 36unusual giomas

Imaging

bull Highly variable imagingcharacters

bull No special typicalimaging criteria

bull Enhancement patternsare highly variable

bull Iso to hypointense onT1WI

bull Heterogenous andhyperintense on T2WI

bull Can contain solid cyticcalcified components

10202016 37unusual giomas

bull FDG PET scan showshypometabolism oreumetabolism in thesepatients

10202016 38unusual giomas

Management And Outcome

bull Gold standard is grosstotal resection

bull Seizure control aftersurgery is as high as80

bull Survival rates are highbull Event free survival of

low grade and highgrade gangiogioma are57 vs 15

bull The optimal surgicalresection depends uponthe eloquent areasinvolved in the vicinityof the tumor

bull The use ofchemotherapy is notwell established

bull Typically they shouldbe allowed for chemoand radiotherapy

10202016 39unusual giomas

bull The use of adjuvant therapy is warranted in thosecases which harbour residual lesions or features ofanaplasia

bull Potential harm to nearby brain areas preclude thejudicious use of radiation

10202016 40unusual giomas

bull Brain Tumor Res Treat 2014 Oct 2(2) 49ndash55

bull Treatment and Outcomes for Gangliogliomas A Single-Center Review of 16 Patients

bull Jye Young Song Jeong Hoon Kim Young Hyun Cho Chang Jin Kim and Eun Jung Lee

bull Ganglioglioma is a rare and slowly growing benign tumor We investigated the outcomes of patients who underwent different combination treatments

bull Methods

bull Between 1998 and 2012 16 patients including 11 men and 5 women with a median age of 125 years (range 25-65 years) were treated for intracranial gangliogliomas at our institution The median follow-up period was 57 years (range 48 days-156 years) Fifteen cases were included in the outcome assessment because one patient was lost to follow-up Complete resection was achieved in 8 (53) patients Six (40) patients underwent incomplete resection with or without adjuvant radiotherapy and one patient with a brainstem tumor underwent only stereotactic biopsy

bull Results

bull Gangliogliomas predominantly affected young (875) male patients and most frequently presented with seizures (64) Of eight patients who underwent complete resection seven did not show recurrence whereas only three of six with incomplete resection showed no recurrence Four patients with recurrence received salvage treatments (two repeat surgeries and two radiosurgeries) A tumor control rate of 93 (1415) was achieved at the last follow-up No recurrence or malignant changes were observed after a median follow-up of 12 and 45 years in four patients who received gamma knife (GK) radiosurgery as adjuvant and salvage treatment

bull Conclusion

bull Complete resection produced the best outcomes and incomplete resection followed by adjuvant or salvage treatments showed favorable outcomes In patients who are not eligible for complete resection because of tumor location or potential neurologic deficits following surgery GK radiosurgery should be considered for the treatment of residual or recurrent tumors

10202016 41unusual giomas

Papillary tumours of pineal

bull WHO grade II AND IIIbull Relatively new edition to WHObull They are equal in both sexesbull Reach medical attention due to headache mental

status changes and altered visionbull Contain eosinophilic cells with distinctive

borders with large pleomorphic nucleibull Positive for cytokeratin synaptophysin stain

variably for GFAP vimentin S-100

10202016 unusual giomas 42

Imaging

bull Generally found to be diffusely enhancinglesion of pineal region

bull Post 3rd ventricle with HCPbull Appear well demarcated from thalamus and

cerebellum

10202016 unusual giomas 43

Mangement

bull Standard treatment is excisionbull Grow slowly and donot transform to

malignant tumorsbull Local recurrence is however possiblebull Radiotherapy appears to provide good control

in local recurrence

10202016 unusual giomas 44

Choroid Glioma Of Third Ventricle

bull WHO Grade IIbull Ist recognised by WHO in

1998bull Arise in the anterior third

ventriclebull Commonly come to

attention after HCPbull Appera to contain in 3rd

ventricle and thought toarise from ventricularsurface

bull Composed of GFAP andvimentin positive cells inmucinous matrix

bull Nuclei are uniform insize and shape

bull Indolent growthbull May show EGF receptor

positivitybull Some suggets these tumor

arise from the lminaterminalis andventricular tanycytes

10202016 45unusual giomas

Imaging

bull Commonly fill 3rd

ventriclebull Diffusely enhance with

contrastbull Appear to infiltrate the

thalamusbull Large tumors may be

difficult to differentiatefrom suprasellar masses

10202016 46unusual giomas

10202016 47unusual giomas

bull Surgical management is limited by locationbull Access through the dialted ventricle and lamina

terminalis may be challangingbull Subtotal resection is thus commonbull Role of adjuvant radiotherapy is unknown

10202016 48unusual giomas

bull Acta Neurochir (Wien) 2008 Nov150(11)1183-91 doi 101007s00701-008-0014-6 Epub

2008 Oct 21

bull Chordoid glioma of the third ventricle

bull Vanhauwaert DJ1 Clement F Van Dorpe J Deruytter MJKGROUND

bull Chordoid glioma is a rare tumour (World Health Organisation grade II) originating from the third ventricle

with both glial and chordoid features It was first described by Brat in 1998 Since there is no detailed

information available on the outcome after surgery and adjuvant treatment we reviewed the literature

bull METHODS

bull A literature search through PUBMED revealed 50 cases of chordoid glioma Most reports

were found in pathology journals Information on the postoperative course was sometimes

very limited We reviewed the available literature and studied in detail the presenting

symptoms mortality and postoperative complications in relation to the extent of resective

surgery as well as the importance of adjuvant treatment

bull CONCLUSIONS

bull Mortality in the immediate postoperative period is 32 and is higher after gross total

resection as compared to subtotal resection Non-fatal postoperative complications are

hypothalamic disorders and mental alterations Gross total resection is the treatment of

choice since no recurrence has been reported after macroscopically complete resection

but this is often difficult because of the location and adherence to the hypothalamus The

role of postoperative radiotherapy is uncertain There is some indication that radiosurgery

with or without conventional irradiation is superior to conventional radiation alone Planned

subtotal resection followed by stereotactic radiosurgery can be a safe and effective

alternative in a patient in whom gross total resection is considered to be too risky

There is no report on the use of chemotherapy in the treatment of chordoid gliomas More

information about the optimal treatment strategy is needed and more reports are also needed10202016 49unusual giomas

bull Neurologist 2011 Jan17(1)52bull Chordoid glioma a case report and literature reviewbull Liu WP1 Cheng JX Yi XC Zhen HN Fei Z Li Q Zhang Xbull chordoid glioma is a rare tumor (World Health Organization grade II) with both

glial and chordoid features often located in the suprasellar region and anterior third ventricle It was first described by Brat in 1998 Because there is no detailed information available from the clinical perspective we reviewed the literature

bull METHODS

bull a literature search through PUBMED and CNKI revealed 64 cases of chordoid glioma Information on the clinical course was very limited We reviewed the literature and studied the pathologic and imaging features postoperative mortality and morbidity in relation to surgical extension and approaches and the importance of adjuvant treatment

bull CONCLUSIONS bull mortality in the immediate postoperative period is 28 and postoperative

morbidity is 60 which are statistically higher after gross total resection as compared with subtotal resection Translamina terminalis approach is considered to be the best approach The current study cannot document that patients have longer survival and higher quality of life after gross total resection than subtotal resection The role of postoperative radiotherapy is uncertain and there is no report on the use of chemotherapy More information about the optimal treatment strategy is needed

10202016 50unusual giomas

Conclusion

bull They are identified by their unique morphologicalfeatures

bull Many of these have indolent course and identified byepilepsy

bull Potentail diagnosis should be considered in youngpatients with seizures and cortically based lesions

bull Correct diagnosis requires experiencedneuropathologisrt

bull As more experienced is gained more knowledgetowards better understanding shall provide betteroutcomes in these patients

10202016 unusual giomas 51

10202016 52unusual giomas

Content supportndash youmann neurosurgery textbook

bull Supratentorail are mostoften cortical and meningesbased

bull Chronic seizuresbull Grossely these are firm

tumors variable in colorrelatively avascular

bull Typically invade the piaarachnoid space

bull 13 pts have involvementof all the three meninges

bull Cyst fluid is xanthochromic

bull Very specific microscpoiccriteria for diagnosis are

1 Pleomorphic andxanthomatous astrocytes

2 Perivascular lymphocytes3 Non infilterating histology4 Reticulin stainingbull Pleomorphism can be as

vast as glioblastoma orsarcomatous tumors

10202016 26unusual giomas

bull IHC Staining revealsspindle cells and roundpleomorphic cellspositive for S-100 andGFAP

bull Staining forsynaptophysin and NSEmay be also present

bull Some workers havereported them to be asubtype of ganglioglioma

bull Association between theNeurofibromatosis Sturge Weber Syndrome

bull Loss of chr 9 and gain ofchromosome 7 have beenfound

10202016 27unusual giomas

Imaging

bull CTbull PXAs are typically hypo

or isodense and may bewell or poorlydemarcated usually withno or little surroundingoedema

bull Calcification is rarebull Due to its superficial

location it may causescalloping of theoverlying bone

10202016 28unusual giomas

bull MRIbull T1

ndash solid component iso tohypointense cf grey matter

ndash cystic component low signalndash leptomeningeal involvement

seen in over 70 of casesbull T1 C+ (Gd)

ndash solid component usuallyenhances vividly

bull T2ndash solid component iso to

hyperintense cf grey matterndash cystic component high signalndash on T2 FLAIR sequence cystic

areas show hyperintensityrelative to CSF due to higherprotein contents

ndash little surrounding vasogenicoedema

10202016 29unusual giomas

Management amp Outcome

bull Quite rare tumorsbull Surgical excision appears to

be best favoured modalitybull Actuarial survival trates are

91 at 5 years and 82 at10 years

bull Median survival 18 years

bull Total resection should beattempted in every case asfar as possible

bull Resection of cyst lining isnot indicated

bull Data regarding the use ofadjuvant therapy at thistime are limited to few casereports only

bull Pts who had incompleteresection high mitoticindex necrosis should bereserved for strict vigilanceand CEMRI on follow ups

10202016 30unusual giomas

bull At progression repeatresection be attempted

bull Proression should betaken as aggressivetumor type

bull Malignanttransformation maynecessitate adjuvanttherapy

10202016 31unusual giomas

bull Br J Neurosurg 2013 Dec27(6)759-64 doi 103109026886972013776666 Epub 2013 Mar 20

bull Pleomorphic xanthoastrocytoma long-term results of surgical treatment and analysis of prognosticfactors

bull Gallo P1 Cecchi PC Locatelli F Rizzo P Ghimenton C Gerosa M Pinna G

bull BACKGROUND

bull Pleomorphic Xanthoastrocytoma (PXA) is a rare brain tumour most commonly affecting children andyoung adults To date only few data regarding the long-term follow-up of these patients after surgery are

available The aim of this study is to describe our single-institution experience in the surgicalmanagement of this particular glioma over a period of over 18 years

bull METHODS

bull We performed a retrospective review of all cases of PXA (40 patients) operated upon at theDepartment of Neurosurgery of Verona Italy between 1990 and 2008 The impact of clinical radiologicalsurgical and histological factors on overall survival (OS) and progression-free survival (PFS) was analysed bymeans of univariate and multivariate models

bull FINDINGS

bull We achieved a gross total resection (GTR) in 65 of patients Histological diagnosis was of grade II in 80anaplastic features were present in the remaining 20 Adjuvant treatment radiotherapy or chemo-

radiotherapy was administered in 40 of the cases Median follow-up was 74 months OS at 5- and10 years was 7632 and 6824 respectively PFS at 5- and 10 years was 71 and 58respectively In the multivariate model histological grade extent of resection and age at diagnosis (le 30years vs gt 30 years) were the only independent prognostic factors for both OS and PFS

bull CONCLUSIONS

bull Our retrospective long-term study confirms the relatively favourable prognosis associatedwith PXA Young patients with a low-grade tumour (WHO grade II) who underwent GTR carrythe longest OS and PFS

10202016 32unusual giomas

bull Brain Tumor Pathol 2006 Apr23(1)55-63

bull A case of anaplastic pleomorphic xanthoastrocytoma presenting with tumor bleeding and cerebrospinal fluid dissemination

bull Asano K1 Miyamoto S Kubo O Kikkukawa T Yagihashi A Ohkuma H

bull Pleomorphic xanthoastrocytoma (PXA) has been considered an astrocytic tumorwith a relatively favorable prognosis However PXA cases having several recurrent patterns with poor prognosis have been reported in recent years and a new concept of anaplastic PXA has been proposed The present case was a 59-year-old woman who presented with tumor bleeding onset and cerebrospinal fluid dissemination The patient had sudden-onset right hemiparesis aphasia and consciousness disturbance and was admitted to a local area hospital After emergency surgery had removed the hematoma postoperative contrast-enhanced CT scan revealed a left temporal tumor A second surgery was therefore performed for initial tumor removal 2 months later Histopathological findings showed that the tumor was typical PXA with strong pleomorphism and xanthomatous changes and contained an ependymoma-like component in the center area However endothelial proliferation and mitosis were more remarkable compared to ordinary PXA The MIB-1 labeling index was 98 high From these findings the histopathological diagnosis was anaplastic PXA The patient underwent surgery to remove recurrent tumors 5 and 16 months later The patient died 36 months after the first onset and CT revealed glioblastoma-like

findings and cerebrospinal fluid dissemination This case report is the first case in which PXA presented with tumor bleeding onset Histopathological findings suggested anaplastic PXA from the first surgical specimens and PXA recurred many times We thus believe that the patient displayed primary anaplastic PXA rather than secondary anaplastic PXA that results in malignant transformation10202016 33unusual giomas

Ganglioglioma

bull WHO grade 1bull Initially described by

Perkins in 1926bull Benign well

differentiatedneuroepithelial tumors

bull Most commonly found intemporal lobe though canoccur anywhere in CNS

bull Commonly found inyoung patients withepilepsy

bull Most commonly notedtumor in temporal lobeepilepsy patients

bull Have been described inbrain steem spinal cordand cerebellum

bull Account for 1 ofprimary brain tumors

10202016 34unusual giomas

bull Male dominencebull Most common features

are seizures headachedizziness ataxiaprogressive weakness

bull Malignanttransformation is rare

bull Histology showsimmature mixture ofabnormal neural andglial elements

bull Macroscopically appearsolid or cystic

bull Cystic are welldelineatedfromsurrounding brain

bull Calcification is common

10202016 35unusual giomas

bull IHC staing shows reactivity for GFAP S-100 andvimentin

bull Neurons show synaptophysin class III betatubulin NFP chromogranin reactivity

10202016 36unusual giomas

Imaging

bull Highly variable imagingcharacters

bull No special typicalimaging criteria

bull Enhancement patternsare highly variable

bull Iso to hypointense onT1WI

bull Heterogenous andhyperintense on T2WI

bull Can contain solid cyticcalcified components

10202016 37unusual giomas

bull FDG PET scan showshypometabolism oreumetabolism in thesepatients

10202016 38unusual giomas

Management And Outcome

bull Gold standard is grosstotal resection

bull Seizure control aftersurgery is as high as80

bull Survival rates are highbull Event free survival of

low grade and highgrade gangiogioma are57 vs 15

bull The optimal surgicalresection depends uponthe eloquent areasinvolved in the vicinityof the tumor

bull The use ofchemotherapy is notwell established

bull Typically they shouldbe allowed for chemoand radiotherapy

10202016 39unusual giomas

bull The use of adjuvant therapy is warranted in thosecases which harbour residual lesions or features ofanaplasia

bull Potential harm to nearby brain areas preclude thejudicious use of radiation

10202016 40unusual giomas

bull Brain Tumor Res Treat 2014 Oct 2(2) 49ndash55

bull Treatment and Outcomes for Gangliogliomas A Single-Center Review of 16 Patients

bull Jye Young Song Jeong Hoon Kim Young Hyun Cho Chang Jin Kim and Eun Jung Lee

bull Ganglioglioma is a rare and slowly growing benign tumor We investigated the outcomes of patients who underwent different combination treatments

bull Methods

bull Between 1998 and 2012 16 patients including 11 men and 5 women with a median age of 125 years (range 25-65 years) were treated for intracranial gangliogliomas at our institution The median follow-up period was 57 years (range 48 days-156 years) Fifteen cases were included in the outcome assessment because one patient was lost to follow-up Complete resection was achieved in 8 (53) patients Six (40) patients underwent incomplete resection with or without adjuvant radiotherapy and one patient with a brainstem tumor underwent only stereotactic biopsy

bull Results

bull Gangliogliomas predominantly affected young (875) male patients and most frequently presented with seizures (64) Of eight patients who underwent complete resection seven did not show recurrence whereas only three of six with incomplete resection showed no recurrence Four patients with recurrence received salvage treatments (two repeat surgeries and two radiosurgeries) A tumor control rate of 93 (1415) was achieved at the last follow-up No recurrence or malignant changes were observed after a median follow-up of 12 and 45 years in four patients who received gamma knife (GK) radiosurgery as adjuvant and salvage treatment

bull Conclusion

bull Complete resection produced the best outcomes and incomplete resection followed by adjuvant or salvage treatments showed favorable outcomes In patients who are not eligible for complete resection because of tumor location or potential neurologic deficits following surgery GK radiosurgery should be considered for the treatment of residual or recurrent tumors

10202016 41unusual giomas

Papillary tumours of pineal

bull WHO grade II AND IIIbull Relatively new edition to WHObull They are equal in both sexesbull Reach medical attention due to headache mental

status changes and altered visionbull Contain eosinophilic cells with distinctive

borders with large pleomorphic nucleibull Positive for cytokeratin synaptophysin stain

variably for GFAP vimentin S-100

10202016 unusual giomas 42

Imaging

bull Generally found to be diffusely enhancinglesion of pineal region

bull Post 3rd ventricle with HCPbull Appear well demarcated from thalamus and

cerebellum

10202016 unusual giomas 43

Mangement

bull Standard treatment is excisionbull Grow slowly and donot transform to

malignant tumorsbull Local recurrence is however possiblebull Radiotherapy appears to provide good control

in local recurrence

10202016 unusual giomas 44

Choroid Glioma Of Third Ventricle

bull WHO Grade IIbull Ist recognised by WHO in

1998bull Arise in the anterior third

ventriclebull Commonly come to

attention after HCPbull Appera to contain in 3rd

ventricle and thought toarise from ventricularsurface

bull Composed of GFAP andvimentin positive cells inmucinous matrix

bull Nuclei are uniform insize and shape

bull Indolent growthbull May show EGF receptor

positivitybull Some suggets these tumor

arise from the lminaterminalis andventricular tanycytes

10202016 45unusual giomas

Imaging

bull Commonly fill 3rd

ventriclebull Diffusely enhance with

contrastbull Appear to infiltrate the

thalamusbull Large tumors may be

difficult to differentiatefrom suprasellar masses

10202016 46unusual giomas

10202016 47unusual giomas

bull Surgical management is limited by locationbull Access through the dialted ventricle and lamina

terminalis may be challangingbull Subtotal resection is thus commonbull Role of adjuvant radiotherapy is unknown

10202016 48unusual giomas

bull Acta Neurochir (Wien) 2008 Nov150(11)1183-91 doi 101007s00701-008-0014-6 Epub

2008 Oct 21

bull Chordoid glioma of the third ventricle

bull Vanhauwaert DJ1 Clement F Van Dorpe J Deruytter MJKGROUND

bull Chordoid glioma is a rare tumour (World Health Organisation grade II) originating from the third ventricle

with both glial and chordoid features It was first described by Brat in 1998 Since there is no detailed

information available on the outcome after surgery and adjuvant treatment we reviewed the literature

bull METHODS

bull A literature search through PUBMED revealed 50 cases of chordoid glioma Most reports

were found in pathology journals Information on the postoperative course was sometimes

very limited We reviewed the available literature and studied in detail the presenting

symptoms mortality and postoperative complications in relation to the extent of resective

surgery as well as the importance of adjuvant treatment

bull CONCLUSIONS

bull Mortality in the immediate postoperative period is 32 and is higher after gross total

resection as compared to subtotal resection Non-fatal postoperative complications are

hypothalamic disorders and mental alterations Gross total resection is the treatment of

choice since no recurrence has been reported after macroscopically complete resection

but this is often difficult because of the location and adherence to the hypothalamus The

role of postoperative radiotherapy is uncertain There is some indication that radiosurgery

with or without conventional irradiation is superior to conventional radiation alone Planned

subtotal resection followed by stereotactic radiosurgery can be a safe and effective

alternative in a patient in whom gross total resection is considered to be too risky

There is no report on the use of chemotherapy in the treatment of chordoid gliomas More

information about the optimal treatment strategy is needed and more reports are also needed10202016 49unusual giomas

bull Neurologist 2011 Jan17(1)52bull Chordoid glioma a case report and literature reviewbull Liu WP1 Cheng JX Yi XC Zhen HN Fei Z Li Q Zhang Xbull chordoid glioma is a rare tumor (World Health Organization grade II) with both

glial and chordoid features often located in the suprasellar region and anterior third ventricle It was first described by Brat in 1998 Because there is no detailed information available from the clinical perspective we reviewed the literature

bull METHODS

bull a literature search through PUBMED and CNKI revealed 64 cases of chordoid glioma Information on the clinical course was very limited We reviewed the literature and studied the pathologic and imaging features postoperative mortality and morbidity in relation to surgical extension and approaches and the importance of adjuvant treatment

bull CONCLUSIONS bull mortality in the immediate postoperative period is 28 and postoperative

morbidity is 60 which are statistically higher after gross total resection as compared with subtotal resection Translamina terminalis approach is considered to be the best approach The current study cannot document that patients have longer survival and higher quality of life after gross total resection than subtotal resection The role of postoperative radiotherapy is uncertain and there is no report on the use of chemotherapy More information about the optimal treatment strategy is needed

10202016 50unusual giomas

Conclusion

bull They are identified by their unique morphologicalfeatures

bull Many of these have indolent course and identified byepilepsy

bull Potentail diagnosis should be considered in youngpatients with seizures and cortically based lesions

bull Correct diagnosis requires experiencedneuropathologisrt

bull As more experienced is gained more knowledgetowards better understanding shall provide betteroutcomes in these patients

10202016 unusual giomas 51

10202016 52unusual giomas

Content supportndash youmann neurosurgery textbook

bull IHC Staining revealsspindle cells and roundpleomorphic cellspositive for S-100 andGFAP

bull Staining forsynaptophysin and NSEmay be also present

bull Some workers havereported them to be asubtype of ganglioglioma

bull Association between theNeurofibromatosis Sturge Weber Syndrome

bull Loss of chr 9 and gain ofchromosome 7 have beenfound

10202016 27unusual giomas

Imaging

bull CTbull PXAs are typically hypo

or isodense and may bewell or poorlydemarcated usually withno or little surroundingoedema

bull Calcification is rarebull Due to its superficial

location it may causescalloping of theoverlying bone

10202016 28unusual giomas

bull MRIbull T1

ndash solid component iso tohypointense cf grey matter

ndash cystic component low signalndash leptomeningeal involvement

seen in over 70 of casesbull T1 C+ (Gd)

ndash solid component usuallyenhances vividly

bull T2ndash solid component iso to

hyperintense cf grey matterndash cystic component high signalndash on T2 FLAIR sequence cystic

areas show hyperintensityrelative to CSF due to higherprotein contents

ndash little surrounding vasogenicoedema

10202016 29unusual giomas

Management amp Outcome

bull Quite rare tumorsbull Surgical excision appears to

be best favoured modalitybull Actuarial survival trates are

91 at 5 years and 82 at10 years

bull Median survival 18 years

bull Total resection should beattempted in every case asfar as possible

bull Resection of cyst lining isnot indicated

bull Data regarding the use ofadjuvant therapy at thistime are limited to few casereports only

bull Pts who had incompleteresection high mitoticindex necrosis should bereserved for strict vigilanceand CEMRI on follow ups

10202016 30unusual giomas

bull At progression repeatresection be attempted

bull Proression should betaken as aggressivetumor type

bull Malignanttransformation maynecessitate adjuvanttherapy

10202016 31unusual giomas

bull Br J Neurosurg 2013 Dec27(6)759-64 doi 103109026886972013776666 Epub 2013 Mar 20

bull Pleomorphic xanthoastrocytoma long-term results of surgical treatment and analysis of prognosticfactors

bull Gallo P1 Cecchi PC Locatelli F Rizzo P Ghimenton C Gerosa M Pinna G

bull BACKGROUND

bull Pleomorphic Xanthoastrocytoma (PXA) is a rare brain tumour most commonly affecting children andyoung adults To date only few data regarding the long-term follow-up of these patients after surgery are

available The aim of this study is to describe our single-institution experience in the surgicalmanagement of this particular glioma over a period of over 18 years

bull METHODS

bull We performed a retrospective review of all cases of PXA (40 patients) operated upon at theDepartment of Neurosurgery of Verona Italy between 1990 and 2008 The impact of clinical radiologicalsurgical and histological factors on overall survival (OS) and progression-free survival (PFS) was analysed bymeans of univariate and multivariate models

bull FINDINGS

bull We achieved a gross total resection (GTR) in 65 of patients Histological diagnosis was of grade II in 80anaplastic features were present in the remaining 20 Adjuvant treatment radiotherapy or chemo-

radiotherapy was administered in 40 of the cases Median follow-up was 74 months OS at 5- and10 years was 7632 and 6824 respectively PFS at 5- and 10 years was 71 and 58respectively In the multivariate model histological grade extent of resection and age at diagnosis (le 30years vs gt 30 years) were the only independent prognostic factors for both OS and PFS

bull CONCLUSIONS

bull Our retrospective long-term study confirms the relatively favourable prognosis associatedwith PXA Young patients with a low-grade tumour (WHO grade II) who underwent GTR carrythe longest OS and PFS

10202016 32unusual giomas

bull Brain Tumor Pathol 2006 Apr23(1)55-63

bull A case of anaplastic pleomorphic xanthoastrocytoma presenting with tumor bleeding and cerebrospinal fluid dissemination

bull Asano K1 Miyamoto S Kubo O Kikkukawa T Yagihashi A Ohkuma H

bull Pleomorphic xanthoastrocytoma (PXA) has been considered an astrocytic tumorwith a relatively favorable prognosis However PXA cases having several recurrent patterns with poor prognosis have been reported in recent years and a new concept of anaplastic PXA has been proposed The present case was a 59-year-old woman who presented with tumor bleeding onset and cerebrospinal fluid dissemination The patient had sudden-onset right hemiparesis aphasia and consciousness disturbance and was admitted to a local area hospital After emergency surgery had removed the hematoma postoperative contrast-enhanced CT scan revealed a left temporal tumor A second surgery was therefore performed for initial tumor removal 2 months later Histopathological findings showed that the tumor was typical PXA with strong pleomorphism and xanthomatous changes and contained an ependymoma-like component in the center area However endothelial proliferation and mitosis were more remarkable compared to ordinary PXA The MIB-1 labeling index was 98 high From these findings the histopathological diagnosis was anaplastic PXA The patient underwent surgery to remove recurrent tumors 5 and 16 months later The patient died 36 months after the first onset and CT revealed glioblastoma-like

findings and cerebrospinal fluid dissemination This case report is the first case in which PXA presented with tumor bleeding onset Histopathological findings suggested anaplastic PXA from the first surgical specimens and PXA recurred many times We thus believe that the patient displayed primary anaplastic PXA rather than secondary anaplastic PXA that results in malignant transformation10202016 33unusual giomas

Ganglioglioma

bull WHO grade 1bull Initially described by

Perkins in 1926bull Benign well

differentiatedneuroepithelial tumors

bull Most commonly found intemporal lobe though canoccur anywhere in CNS

bull Commonly found inyoung patients withepilepsy

bull Most commonly notedtumor in temporal lobeepilepsy patients

bull Have been described inbrain steem spinal cordand cerebellum

bull Account for 1 ofprimary brain tumors

10202016 34unusual giomas

bull Male dominencebull Most common features

are seizures headachedizziness ataxiaprogressive weakness

bull Malignanttransformation is rare

bull Histology showsimmature mixture ofabnormal neural andglial elements

bull Macroscopically appearsolid or cystic

bull Cystic are welldelineatedfromsurrounding brain

bull Calcification is common

10202016 35unusual giomas

bull IHC staing shows reactivity for GFAP S-100 andvimentin

bull Neurons show synaptophysin class III betatubulin NFP chromogranin reactivity

10202016 36unusual giomas

Imaging

bull Highly variable imagingcharacters

bull No special typicalimaging criteria

bull Enhancement patternsare highly variable

bull Iso to hypointense onT1WI

bull Heterogenous andhyperintense on T2WI

bull Can contain solid cyticcalcified components

10202016 37unusual giomas

bull FDG PET scan showshypometabolism oreumetabolism in thesepatients

10202016 38unusual giomas

Management And Outcome

bull Gold standard is grosstotal resection

bull Seizure control aftersurgery is as high as80

bull Survival rates are highbull Event free survival of

low grade and highgrade gangiogioma are57 vs 15

bull The optimal surgicalresection depends uponthe eloquent areasinvolved in the vicinityof the tumor

bull The use ofchemotherapy is notwell established

bull Typically they shouldbe allowed for chemoand radiotherapy

10202016 39unusual giomas

bull The use of adjuvant therapy is warranted in thosecases which harbour residual lesions or features ofanaplasia

bull Potential harm to nearby brain areas preclude thejudicious use of radiation

10202016 40unusual giomas

bull Brain Tumor Res Treat 2014 Oct 2(2) 49ndash55

bull Treatment and Outcomes for Gangliogliomas A Single-Center Review of 16 Patients

bull Jye Young Song Jeong Hoon Kim Young Hyun Cho Chang Jin Kim and Eun Jung Lee

bull Ganglioglioma is a rare and slowly growing benign tumor We investigated the outcomes of patients who underwent different combination treatments

bull Methods

bull Between 1998 and 2012 16 patients including 11 men and 5 women with a median age of 125 years (range 25-65 years) were treated for intracranial gangliogliomas at our institution The median follow-up period was 57 years (range 48 days-156 years) Fifteen cases were included in the outcome assessment because one patient was lost to follow-up Complete resection was achieved in 8 (53) patients Six (40) patients underwent incomplete resection with or without adjuvant radiotherapy and one patient with a brainstem tumor underwent only stereotactic biopsy

bull Results

bull Gangliogliomas predominantly affected young (875) male patients and most frequently presented with seizures (64) Of eight patients who underwent complete resection seven did not show recurrence whereas only three of six with incomplete resection showed no recurrence Four patients with recurrence received salvage treatments (two repeat surgeries and two radiosurgeries) A tumor control rate of 93 (1415) was achieved at the last follow-up No recurrence or malignant changes were observed after a median follow-up of 12 and 45 years in four patients who received gamma knife (GK) radiosurgery as adjuvant and salvage treatment

bull Conclusion

bull Complete resection produced the best outcomes and incomplete resection followed by adjuvant or salvage treatments showed favorable outcomes In patients who are not eligible for complete resection because of tumor location or potential neurologic deficits following surgery GK radiosurgery should be considered for the treatment of residual or recurrent tumors

10202016 41unusual giomas

Papillary tumours of pineal

bull WHO grade II AND IIIbull Relatively new edition to WHObull They are equal in both sexesbull Reach medical attention due to headache mental

status changes and altered visionbull Contain eosinophilic cells with distinctive

borders with large pleomorphic nucleibull Positive for cytokeratin synaptophysin stain

variably for GFAP vimentin S-100

10202016 unusual giomas 42

Imaging

bull Generally found to be diffusely enhancinglesion of pineal region

bull Post 3rd ventricle with HCPbull Appear well demarcated from thalamus and

cerebellum

10202016 unusual giomas 43

Mangement

bull Standard treatment is excisionbull Grow slowly and donot transform to

malignant tumorsbull Local recurrence is however possiblebull Radiotherapy appears to provide good control

in local recurrence

10202016 unusual giomas 44

Choroid Glioma Of Third Ventricle

bull WHO Grade IIbull Ist recognised by WHO in

1998bull Arise in the anterior third

ventriclebull Commonly come to

attention after HCPbull Appera to contain in 3rd

ventricle and thought toarise from ventricularsurface

bull Composed of GFAP andvimentin positive cells inmucinous matrix

bull Nuclei are uniform insize and shape

bull Indolent growthbull May show EGF receptor

positivitybull Some suggets these tumor

arise from the lminaterminalis andventricular tanycytes

10202016 45unusual giomas

Imaging

bull Commonly fill 3rd

ventriclebull Diffusely enhance with

contrastbull Appear to infiltrate the

thalamusbull Large tumors may be

difficult to differentiatefrom suprasellar masses

10202016 46unusual giomas

10202016 47unusual giomas

bull Surgical management is limited by locationbull Access through the dialted ventricle and lamina

terminalis may be challangingbull Subtotal resection is thus commonbull Role of adjuvant radiotherapy is unknown

10202016 48unusual giomas

bull Acta Neurochir (Wien) 2008 Nov150(11)1183-91 doi 101007s00701-008-0014-6 Epub

2008 Oct 21

bull Chordoid glioma of the third ventricle

bull Vanhauwaert DJ1 Clement F Van Dorpe J Deruytter MJKGROUND

bull Chordoid glioma is a rare tumour (World Health Organisation grade II) originating from the third ventricle

with both glial and chordoid features It was first described by Brat in 1998 Since there is no detailed

information available on the outcome after surgery and adjuvant treatment we reviewed the literature

bull METHODS

bull A literature search through PUBMED revealed 50 cases of chordoid glioma Most reports

were found in pathology journals Information on the postoperative course was sometimes

very limited We reviewed the available literature and studied in detail the presenting

symptoms mortality and postoperative complications in relation to the extent of resective

surgery as well as the importance of adjuvant treatment

bull CONCLUSIONS

bull Mortality in the immediate postoperative period is 32 and is higher after gross total

resection as compared to subtotal resection Non-fatal postoperative complications are

hypothalamic disorders and mental alterations Gross total resection is the treatment of

choice since no recurrence has been reported after macroscopically complete resection

but this is often difficult because of the location and adherence to the hypothalamus The

role of postoperative radiotherapy is uncertain There is some indication that radiosurgery

with or without conventional irradiation is superior to conventional radiation alone Planned

subtotal resection followed by stereotactic radiosurgery can be a safe and effective

alternative in a patient in whom gross total resection is considered to be too risky

There is no report on the use of chemotherapy in the treatment of chordoid gliomas More

information about the optimal treatment strategy is needed and more reports are also needed10202016 49unusual giomas

bull Neurologist 2011 Jan17(1)52bull Chordoid glioma a case report and literature reviewbull Liu WP1 Cheng JX Yi XC Zhen HN Fei Z Li Q Zhang Xbull chordoid glioma is a rare tumor (World Health Organization grade II) with both

glial and chordoid features often located in the suprasellar region and anterior third ventricle It was first described by Brat in 1998 Because there is no detailed information available from the clinical perspective we reviewed the literature

bull METHODS

bull a literature search through PUBMED and CNKI revealed 64 cases of chordoid glioma Information on the clinical course was very limited We reviewed the literature and studied the pathologic and imaging features postoperative mortality and morbidity in relation to surgical extension and approaches and the importance of adjuvant treatment

bull CONCLUSIONS bull mortality in the immediate postoperative period is 28 and postoperative

morbidity is 60 which are statistically higher after gross total resection as compared with subtotal resection Translamina terminalis approach is considered to be the best approach The current study cannot document that patients have longer survival and higher quality of life after gross total resection than subtotal resection The role of postoperative radiotherapy is uncertain and there is no report on the use of chemotherapy More information about the optimal treatment strategy is needed

10202016 50unusual giomas

Conclusion

bull They are identified by their unique morphologicalfeatures

bull Many of these have indolent course and identified byepilepsy

bull Potentail diagnosis should be considered in youngpatients with seizures and cortically based lesions

bull Correct diagnosis requires experiencedneuropathologisrt

bull As more experienced is gained more knowledgetowards better understanding shall provide betteroutcomes in these patients

10202016 unusual giomas 51

10202016 52unusual giomas

Content supportndash youmann neurosurgery textbook

Imaging

bull CTbull PXAs are typically hypo

or isodense and may bewell or poorlydemarcated usually withno or little surroundingoedema

bull Calcification is rarebull Due to its superficial

location it may causescalloping of theoverlying bone

10202016 28unusual giomas

bull MRIbull T1

ndash solid component iso tohypointense cf grey matter

ndash cystic component low signalndash leptomeningeal involvement

seen in over 70 of casesbull T1 C+ (Gd)

ndash solid component usuallyenhances vividly

bull T2ndash solid component iso to

hyperintense cf grey matterndash cystic component high signalndash on T2 FLAIR sequence cystic

areas show hyperintensityrelative to CSF due to higherprotein contents

ndash little surrounding vasogenicoedema

10202016 29unusual giomas

Management amp Outcome

bull Quite rare tumorsbull Surgical excision appears to

be best favoured modalitybull Actuarial survival trates are

91 at 5 years and 82 at10 years

bull Median survival 18 years

bull Total resection should beattempted in every case asfar as possible

bull Resection of cyst lining isnot indicated

bull Data regarding the use ofadjuvant therapy at thistime are limited to few casereports only

bull Pts who had incompleteresection high mitoticindex necrosis should bereserved for strict vigilanceand CEMRI on follow ups

10202016 30unusual giomas

bull At progression repeatresection be attempted

bull Proression should betaken as aggressivetumor type

bull Malignanttransformation maynecessitate adjuvanttherapy

10202016 31unusual giomas

bull Br J Neurosurg 2013 Dec27(6)759-64 doi 103109026886972013776666 Epub 2013 Mar 20

bull Pleomorphic xanthoastrocytoma long-term results of surgical treatment and analysis of prognosticfactors

bull Gallo P1 Cecchi PC Locatelli F Rizzo P Ghimenton C Gerosa M Pinna G

bull BACKGROUND

bull Pleomorphic Xanthoastrocytoma (PXA) is a rare brain tumour most commonly affecting children andyoung adults To date only few data regarding the long-term follow-up of these patients after surgery are

available The aim of this study is to describe our single-institution experience in the surgicalmanagement of this particular glioma over a period of over 18 years

bull METHODS

bull We performed a retrospective review of all cases of PXA (40 patients) operated upon at theDepartment of Neurosurgery of Verona Italy between 1990 and 2008 The impact of clinical radiologicalsurgical and histological factors on overall survival (OS) and progression-free survival (PFS) was analysed bymeans of univariate and multivariate models

bull FINDINGS

bull We achieved a gross total resection (GTR) in 65 of patients Histological diagnosis was of grade II in 80anaplastic features were present in the remaining 20 Adjuvant treatment radiotherapy or chemo-

radiotherapy was administered in 40 of the cases Median follow-up was 74 months OS at 5- and10 years was 7632 and 6824 respectively PFS at 5- and 10 years was 71 and 58respectively In the multivariate model histological grade extent of resection and age at diagnosis (le 30years vs gt 30 years) were the only independent prognostic factors for both OS and PFS

bull CONCLUSIONS

bull Our retrospective long-term study confirms the relatively favourable prognosis associatedwith PXA Young patients with a low-grade tumour (WHO grade II) who underwent GTR carrythe longest OS and PFS

10202016 32unusual giomas

bull Brain Tumor Pathol 2006 Apr23(1)55-63

bull A case of anaplastic pleomorphic xanthoastrocytoma presenting with tumor bleeding and cerebrospinal fluid dissemination

bull Asano K1 Miyamoto S Kubo O Kikkukawa T Yagihashi A Ohkuma H

bull Pleomorphic xanthoastrocytoma (PXA) has been considered an astrocytic tumorwith a relatively favorable prognosis However PXA cases having several recurrent patterns with poor prognosis have been reported in recent years and a new concept of anaplastic PXA has been proposed The present case was a 59-year-old woman who presented with tumor bleeding onset and cerebrospinal fluid dissemination The patient had sudden-onset right hemiparesis aphasia and consciousness disturbance and was admitted to a local area hospital After emergency surgery had removed the hematoma postoperative contrast-enhanced CT scan revealed a left temporal tumor A second surgery was therefore performed for initial tumor removal 2 months later Histopathological findings showed that the tumor was typical PXA with strong pleomorphism and xanthomatous changes and contained an ependymoma-like component in the center area However endothelial proliferation and mitosis were more remarkable compared to ordinary PXA The MIB-1 labeling index was 98 high From these findings the histopathological diagnosis was anaplastic PXA The patient underwent surgery to remove recurrent tumors 5 and 16 months later The patient died 36 months after the first onset and CT revealed glioblastoma-like

findings and cerebrospinal fluid dissemination This case report is the first case in which PXA presented with tumor bleeding onset Histopathological findings suggested anaplastic PXA from the first surgical specimens and PXA recurred many times We thus believe that the patient displayed primary anaplastic PXA rather than secondary anaplastic PXA that results in malignant transformation10202016 33unusual giomas

Ganglioglioma

bull WHO grade 1bull Initially described by

Perkins in 1926bull Benign well

differentiatedneuroepithelial tumors

bull Most commonly found intemporal lobe though canoccur anywhere in CNS

bull Commonly found inyoung patients withepilepsy

bull Most commonly notedtumor in temporal lobeepilepsy patients

bull Have been described inbrain steem spinal cordand cerebellum

bull Account for 1 ofprimary brain tumors

10202016 34unusual giomas

bull Male dominencebull Most common features

are seizures headachedizziness ataxiaprogressive weakness

bull Malignanttransformation is rare

bull Histology showsimmature mixture ofabnormal neural andglial elements

bull Macroscopically appearsolid or cystic

bull Cystic are welldelineatedfromsurrounding brain

bull Calcification is common

10202016 35unusual giomas

bull IHC staing shows reactivity for GFAP S-100 andvimentin

bull Neurons show synaptophysin class III betatubulin NFP chromogranin reactivity

10202016 36unusual giomas

Imaging

bull Highly variable imagingcharacters

bull No special typicalimaging criteria

bull Enhancement patternsare highly variable

bull Iso to hypointense onT1WI

bull Heterogenous andhyperintense on T2WI

bull Can contain solid cyticcalcified components

10202016 37unusual giomas

bull FDG PET scan showshypometabolism oreumetabolism in thesepatients

10202016 38unusual giomas

Management And Outcome

bull Gold standard is grosstotal resection

bull Seizure control aftersurgery is as high as80

bull Survival rates are highbull Event free survival of

low grade and highgrade gangiogioma are57 vs 15

bull The optimal surgicalresection depends uponthe eloquent areasinvolved in the vicinityof the tumor

bull The use ofchemotherapy is notwell established

bull Typically they shouldbe allowed for chemoand radiotherapy

10202016 39unusual giomas

bull The use of adjuvant therapy is warranted in thosecases which harbour residual lesions or features ofanaplasia

bull Potential harm to nearby brain areas preclude thejudicious use of radiation

10202016 40unusual giomas

bull Brain Tumor Res Treat 2014 Oct 2(2) 49ndash55

bull Treatment and Outcomes for Gangliogliomas A Single-Center Review of 16 Patients

bull Jye Young Song Jeong Hoon Kim Young Hyun Cho Chang Jin Kim and Eun Jung Lee

bull Ganglioglioma is a rare and slowly growing benign tumor We investigated the outcomes of patients who underwent different combination treatments

bull Methods

bull Between 1998 and 2012 16 patients including 11 men and 5 women with a median age of 125 years (range 25-65 years) were treated for intracranial gangliogliomas at our institution The median follow-up period was 57 years (range 48 days-156 years) Fifteen cases were included in the outcome assessment because one patient was lost to follow-up Complete resection was achieved in 8 (53) patients Six (40) patients underwent incomplete resection with or without adjuvant radiotherapy and one patient with a brainstem tumor underwent only stereotactic biopsy

bull Results

bull Gangliogliomas predominantly affected young (875) male patients and most frequently presented with seizures (64) Of eight patients who underwent complete resection seven did not show recurrence whereas only three of six with incomplete resection showed no recurrence Four patients with recurrence received salvage treatments (two repeat surgeries and two radiosurgeries) A tumor control rate of 93 (1415) was achieved at the last follow-up No recurrence or malignant changes were observed after a median follow-up of 12 and 45 years in four patients who received gamma knife (GK) radiosurgery as adjuvant and salvage treatment

bull Conclusion

bull Complete resection produced the best outcomes and incomplete resection followed by adjuvant or salvage treatments showed favorable outcomes In patients who are not eligible for complete resection because of tumor location or potential neurologic deficits following surgery GK radiosurgery should be considered for the treatment of residual or recurrent tumors

10202016 41unusual giomas

Papillary tumours of pineal

bull WHO grade II AND IIIbull Relatively new edition to WHObull They are equal in both sexesbull Reach medical attention due to headache mental

status changes and altered visionbull Contain eosinophilic cells with distinctive

borders with large pleomorphic nucleibull Positive for cytokeratin synaptophysin stain

variably for GFAP vimentin S-100

10202016 unusual giomas 42

Imaging

bull Generally found to be diffusely enhancinglesion of pineal region

bull Post 3rd ventricle with HCPbull Appear well demarcated from thalamus and

cerebellum

10202016 unusual giomas 43

Mangement

bull Standard treatment is excisionbull Grow slowly and donot transform to

malignant tumorsbull Local recurrence is however possiblebull Radiotherapy appears to provide good control

in local recurrence

10202016 unusual giomas 44

Choroid Glioma Of Third Ventricle

bull WHO Grade IIbull Ist recognised by WHO in

1998bull Arise in the anterior third

ventriclebull Commonly come to

attention after HCPbull Appera to contain in 3rd

ventricle and thought toarise from ventricularsurface

bull Composed of GFAP andvimentin positive cells inmucinous matrix

bull Nuclei are uniform insize and shape

bull Indolent growthbull May show EGF receptor

positivitybull Some suggets these tumor

arise from the lminaterminalis andventricular tanycytes

10202016 45unusual giomas

Imaging

bull Commonly fill 3rd

ventriclebull Diffusely enhance with

contrastbull Appear to infiltrate the

thalamusbull Large tumors may be

difficult to differentiatefrom suprasellar masses

10202016 46unusual giomas

10202016 47unusual giomas

bull Surgical management is limited by locationbull Access through the dialted ventricle and lamina

terminalis may be challangingbull Subtotal resection is thus commonbull Role of adjuvant radiotherapy is unknown

10202016 48unusual giomas

bull Acta Neurochir (Wien) 2008 Nov150(11)1183-91 doi 101007s00701-008-0014-6 Epub

2008 Oct 21

bull Chordoid glioma of the third ventricle

bull Vanhauwaert DJ1 Clement F Van Dorpe J Deruytter MJKGROUND

bull Chordoid glioma is a rare tumour (World Health Organisation grade II) originating from the third ventricle

with both glial and chordoid features It was first described by Brat in 1998 Since there is no detailed

information available on the outcome after surgery and adjuvant treatment we reviewed the literature

bull METHODS

bull A literature search through PUBMED revealed 50 cases of chordoid glioma Most reports

were found in pathology journals Information on the postoperative course was sometimes

very limited We reviewed the available literature and studied in detail the presenting

symptoms mortality and postoperative complications in relation to the extent of resective

surgery as well as the importance of adjuvant treatment

bull CONCLUSIONS

bull Mortality in the immediate postoperative period is 32 and is higher after gross total

resection as compared to subtotal resection Non-fatal postoperative complications are

hypothalamic disorders and mental alterations Gross total resection is the treatment of

choice since no recurrence has been reported after macroscopically complete resection

but this is often difficult because of the location and adherence to the hypothalamus The

role of postoperative radiotherapy is uncertain There is some indication that radiosurgery

with or without conventional irradiation is superior to conventional radiation alone Planned

subtotal resection followed by stereotactic radiosurgery can be a safe and effective

alternative in a patient in whom gross total resection is considered to be too risky

There is no report on the use of chemotherapy in the treatment of chordoid gliomas More

information about the optimal treatment strategy is needed and more reports are also needed10202016 49unusual giomas

bull Neurologist 2011 Jan17(1)52bull Chordoid glioma a case report and literature reviewbull Liu WP1 Cheng JX Yi XC Zhen HN Fei Z Li Q Zhang Xbull chordoid glioma is a rare tumor (World Health Organization grade II) with both

glial and chordoid features often located in the suprasellar region and anterior third ventricle It was first described by Brat in 1998 Because there is no detailed information available from the clinical perspective we reviewed the literature

bull METHODS

bull a literature search through PUBMED and CNKI revealed 64 cases of chordoid glioma Information on the clinical course was very limited We reviewed the literature and studied the pathologic and imaging features postoperative mortality and morbidity in relation to surgical extension and approaches and the importance of adjuvant treatment

bull CONCLUSIONS bull mortality in the immediate postoperative period is 28 and postoperative

morbidity is 60 which are statistically higher after gross total resection as compared with subtotal resection Translamina terminalis approach is considered to be the best approach The current study cannot document that patients have longer survival and higher quality of life after gross total resection than subtotal resection The role of postoperative radiotherapy is uncertain and there is no report on the use of chemotherapy More information about the optimal treatment strategy is needed

10202016 50unusual giomas

Conclusion

bull They are identified by their unique morphologicalfeatures

bull Many of these have indolent course and identified byepilepsy

bull Potentail diagnosis should be considered in youngpatients with seizures and cortically based lesions

bull Correct diagnosis requires experiencedneuropathologisrt

bull As more experienced is gained more knowledgetowards better understanding shall provide betteroutcomes in these patients

10202016 unusual giomas 51

10202016 52unusual giomas

Content supportndash youmann neurosurgery textbook

bull MRIbull T1

ndash solid component iso tohypointense cf grey matter

ndash cystic component low signalndash leptomeningeal involvement

seen in over 70 of casesbull T1 C+ (Gd)

ndash solid component usuallyenhances vividly

bull T2ndash solid component iso to

hyperintense cf grey matterndash cystic component high signalndash on T2 FLAIR sequence cystic

areas show hyperintensityrelative to CSF due to higherprotein contents

ndash little surrounding vasogenicoedema

10202016 29unusual giomas

Management amp Outcome

bull Quite rare tumorsbull Surgical excision appears to

be best favoured modalitybull Actuarial survival trates are

91 at 5 years and 82 at10 years

bull Median survival 18 years

bull Total resection should beattempted in every case asfar as possible

bull Resection of cyst lining isnot indicated

bull Data regarding the use ofadjuvant therapy at thistime are limited to few casereports only

bull Pts who had incompleteresection high mitoticindex necrosis should bereserved for strict vigilanceand CEMRI on follow ups

10202016 30unusual giomas

bull At progression repeatresection be attempted

bull Proression should betaken as aggressivetumor type

bull Malignanttransformation maynecessitate adjuvanttherapy

10202016 31unusual giomas

bull Br J Neurosurg 2013 Dec27(6)759-64 doi 103109026886972013776666 Epub 2013 Mar 20

bull Pleomorphic xanthoastrocytoma long-term results of surgical treatment and analysis of prognosticfactors

bull Gallo P1 Cecchi PC Locatelli F Rizzo P Ghimenton C Gerosa M Pinna G

bull BACKGROUND

bull Pleomorphic Xanthoastrocytoma (PXA) is a rare brain tumour most commonly affecting children andyoung adults To date only few data regarding the long-term follow-up of these patients after surgery are

available The aim of this study is to describe our single-institution experience in the surgicalmanagement of this particular glioma over a period of over 18 years

bull METHODS

bull We performed a retrospective review of all cases of PXA (40 patients) operated upon at theDepartment of Neurosurgery of Verona Italy between 1990 and 2008 The impact of clinical radiologicalsurgical and histological factors on overall survival (OS) and progression-free survival (PFS) was analysed bymeans of univariate and multivariate models

bull FINDINGS

bull We achieved a gross total resection (GTR) in 65 of patients Histological diagnosis was of grade II in 80anaplastic features were present in the remaining 20 Adjuvant treatment radiotherapy or chemo-

radiotherapy was administered in 40 of the cases Median follow-up was 74 months OS at 5- and10 years was 7632 and 6824 respectively PFS at 5- and 10 years was 71 and 58respectively In the multivariate model histological grade extent of resection and age at diagnosis (le 30years vs gt 30 years) were the only independent prognostic factors for both OS and PFS

bull CONCLUSIONS

bull Our retrospective long-term study confirms the relatively favourable prognosis associatedwith PXA Young patients with a low-grade tumour (WHO grade II) who underwent GTR carrythe longest OS and PFS

10202016 32unusual giomas

bull Brain Tumor Pathol 2006 Apr23(1)55-63

bull A case of anaplastic pleomorphic xanthoastrocytoma presenting with tumor bleeding and cerebrospinal fluid dissemination

bull Asano K1 Miyamoto S Kubo O Kikkukawa T Yagihashi A Ohkuma H

bull Pleomorphic xanthoastrocytoma (PXA) has been considered an astrocytic tumorwith a relatively favorable prognosis However PXA cases having several recurrent patterns with poor prognosis have been reported in recent years and a new concept of anaplastic PXA has been proposed The present case was a 59-year-old woman who presented with tumor bleeding onset and cerebrospinal fluid dissemination The patient had sudden-onset right hemiparesis aphasia and consciousness disturbance and was admitted to a local area hospital After emergency surgery had removed the hematoma postoperative contrast-enhanced CT scan revealed a left temporal tumor A second surgery was therefore performed for initial tumor removal 2 months later Histopathological findings showed that the tumor was typical PXA with strong pleomorphism and xanthomatous changes and contained an ependymoma-like component in the center area However endothelial proliferation and mitosis were more remarkable compared to ordinary PXA The MIB-1 labeling index was 98 high From these findings the histopathological diagnosis was anaplastic PXA The patient underwent surgery to remove recurrent tumors 5 and 16 months later The patient died 36 months after the first onset and CT revealed glioblastoma-like

findings and cerebrospinal fluid dissemination This case report is the first case in which PXA presented with tumor bleeding onset Histopathological findings suggested anaplastic PXA from the first surgical specimens and PXA recurred many times We thus believe that the patient displayed primary anaplastic PXA rather than secondary anaplastic PXA that results in malignant transformation10202016 33unusual giomas

Ganglioglioma

bull WHO grade 1bull Initially described by

Perkins in 1926bull Benign well

differentiatedneuroepithelial tumors

bull Most commonly found intemporal lobe though canoccur anywhere in CNS

bull Commonly found inyoung patients withepilepsy

bull Most commonly notedtumor in temporal lobeepilepsy patients

bull Have been described inbrain steem spinal cordand cerebellum

bull Account for 1 ofprimary brain tumors

10202016 34unusual giomas

bull Male dominencebull Most common features

are seizures headachedizziness ataxiaprogressive weakness

bull Malignanttransformation is rare

bull Histology showsimmature mixture ofabnormal neural andglial elements

bull Macroscopically appearsolid or cystic

bull Cystic are welldelineatedfromsurrounding brain

bull Calcification is common

10202016 35unusual giomas

bull IHC staing shows reactivity for GFAP S-100 andvimentin

bull Neurons show synaptophysin class III betatubulin NFP chromogranin reactivity

10202016 36unusual giomas

Imaging

bull Highly variable imagingcharacters

bull No special typicalimaging criteria

bull Enhancement patternsare highly variable

bull Iso to hypointense onT1WI

bull Heterogenous andhyperintense on T2WI

bull Can contain solid cyticcalcified components

10202016 37unusual giomas

bull FDG PET scan showshypometabolism oreumetabolism in thesepatients

10202016 38unusual giomas

Management And Outcome

bull Gold standard is grosstotal resection

bull Seizure control aftersurgery is as high as80

bull Survival rates are highbull Event free survival of

low grade and highgrade gangiogioma are57 vs 15

bull The optimal surgicalresection depends uponthe eloquent areasinvolved in the vicinityof the tumor

bull The use ofchemotherapy is notwell established

bull Typically they shouldbe allowed for chemoand radiotherapy

10202016 39unusual giomas

bull The use of adjuvant therapy is warranted in thosecases which harbour residual lesions or features ofanaplasia

bull Potential harm to nearby brain areas preclude thejudicious use of radiation

10202016 40unusual giomas

bull Brain Tumor Res Treat 2014 Oct 2(2) 49ndash55

bull Treatment and Outcomes for Gangliogliomas A Single-Center Review of 16 Patients

bull Jye Young Song Jeong Hoon Kim Young Hyun Cho Chang Jin Kim and Eun Jung Lee

bull Ganglioglioma is a rare and slowly growing benign tumor We investigated the outcomes of patients who underwent different combination treatments

bull Methods

bull Between 1998 and 2012 16 patients including 11 men and 5 women with a median age of 125 years (range 25-65 years) were treated for intracranial gangliogliomas at our institution The median follow-up period was 57 years (range 48 days-156 years) Fifteen cases were included in the outcome assessment because one patient was lost to follow-up Complete resection was achieved in 8 (53) patients Six (40) patients underwent incomplete resection with or without adjuvant radiotherapy and one patient with a brainstem tumor underwent only stereotactic biopsy

bull Results

bull Gangliogliomas predominantly affected young (875) male patients and most frequently presented with seizures (64) Of eight patients who underwent complete resection seven did not show recurrence whereas only three of six with incomplete resection showed no recurrence Four patients with recurrence received salvage treatments (two repeat surgeries and two radiosurgeries) A tumor control rate of 93 (1415) was achieved at the last follow-up No recurrence or malignant changes were observed after a median follow-up of 12 and 45 years in four patients who received gamma knife (GK) radiosurgery as adjuvant and salvage treatment

bull Conclusion

bull Complete resection produced the best outcomes and incomplete resection followed by adjuvant or salvage treatments showed favorable outcomes In patients who are not eligible for complete resection because of tumor location or potential neurologic deficits following surgery GK radiosurgery should be considered for the treatment of residual or recurrent tumors

10202016 41unusual giomas

Papillary tumours of pineal

bull WHO grade II AND IIIbull Relatively new edition to WHObull They are equal in both sexesbull Reach medical attention due to headache mental

status changes and altered visionbull Contain eosinophilic cells with distinctive

borders with large pleomorphic nucleibull Positive for cytokeratin synaptophysin stain

variably for GFAP vimentin S-100

10202016 unusual giomas 42

Imaging

bull Generally found to be diffusely enhancinglesion of pineal region

bull Post 3rd ventricle with HCPbull Appear well demarcated from thalamus and

cerebellum

10202016 unusual giomas 43

Mangement

bull Standard treatment is excisionbull Grow slowly and donot transform to

malignant tumorsbull Local recurrence is however possiblebull Radiotherapy appears to provide good control

in local recurrence

10202016 unusual giomas 44

Choroid Glioma Of Third Ventricle

bull WHO Grade IIbull Ist recognised by WHO in

1998bull Arise in the anterior third

ventriclebull Commonly come to

attention after HCPbull Appera to contain in 3rd

ventricle and thought toarise from ventricularsurface

bull Composed of GFAP andvimentin positive cells inmucinous matrix

bull Nuclei are uniform insize and shape

bull Indolent growthbull May show EGF receptor

positivitybull Some suggets these tumor

arise from the lminaterminalis andventricular tanycytes

10202016 45unusual giomas

Imaging

bull Commonly fill 3rd

ventriclebull Diffusely enhance with

contrastbull Appear to infiltrate the

thalamusbull Large tumors may be

difficult to differentiatefrom suprasellar masses

10202016 46unusual giomas

10202016 47unusual giomas

bull Surgical management is limited by locationbull Access through the dialted ventricle and lamina

terminalis may be challangingbull Subtotal resection is thus commonbull Role of adjuvant radiotherapy is unknown

10202016 48unusual giomas

bull Acta Neurochir (Wien) 2008 Nov150(11)1183-91 doi 101007s00701-008-0014-6 Epub

2008 Oct 21

bull Chordoid glioma of the third ventricle

bull Vanhauwaert DJ1 Clement F Van Dorpe J Deruytter MJKGROUND

bull Chordoid glioma is a rare tumour (World Health Organisation grade II) originating from the third ventricle

with both glial and chordoid features It was first described by Brat in 1998 Since there is no detailed

information available on the outcome after surgery and adjuvant treatment we reviewed the literature

bull METHODS

bull A literature search through PUBMED revealed 50 cases of chordoid glioma Most reports

were found in pathology journals Information on the postoperative course was sometimes

very limited We reviewed the available literature and studied in detail the presenting

symptoms mortality and postoperative complications in relation to the extent of resective

surgery as well as the importance of adjuvant treatment

bull CONCLUSIONS

bull Mortality in the immediate postoperative period is 32 and is higher after gross total

resection as compared to subtotal resection Non-fatal postoperative complications are

hypothalamic disorders and mental alterations Gross total resection is the treatment of

choice since no recurrence has been reported after macroscopically complete resection

but this is often difficult because of the location and adherence to the hypothalamus The

role of postoperative radiotherapy is uncertain There is some indication that radiosurgery

with or without conventional irradiation is superior to conventional radiation alone Planned

subtotal resection followed by stereotactic radiosurgery can be a safe and effective

alternative in a patient in whom gross total resection is considered to be too risky

There is no report on the use of chemotherapy in the treatment of chordoid gliomas More

information about the optimal treatment strategy is needed and more reports are also needed10202016 49unusual giomas

bull Neurologist 2011 Jan17(1)52bull Chordoid glioma a case report and literature reviewbull Liu WP1 Cheng JX Yi XC Zhen HN Fei Z Li Q Zhang Xbull chordoid glioma is a rare tumor (World Health Organization grade II) with both

glial and chordoid features often located in the suprasellar region and anterior third ventricle It was first described by Brat in 1998 Because there is no detailed information available from the clinical perspective we reviewed the literature

bull METHODS

bull a literature search through PUBMED and CNKI revealed 64 cases of chordoid glioma Information on the clinical course was very limited We reviewed the literature and studied the pathologic and imaging features postoperative mortality and morbidity in relation to surgical extension and approaches and the importance of adjuvant treatment

bull CONCLUSIONS bull mortality in the immediate postoperative period is 28 and postoperative

morbidity is 60 which are statistically higher after gross total resection as compared with subtotal resection Translamina terminalis approach is considered to be the best approach The current study cannot document that patients have longer survival and higher quality of life after gross total resection than subtotal resection The role of postoperative radiotherapy is uncertain and there is no report on the use of chemotherapy More information about the optimal treatment strategy is needed

10202016 50unusual giomas

Conclusion

bull They are identified by their unique morphologicalfeatures

bull Many of these have indolent course and identified byepilepsy

bull Potentail diagnosis should be considered in youngpatients with seizures and cortically based lesions

bull Correct diagnosis requires experiencedneuropathologisrt

bull As more experienced is gained more knowledgetowards better understanding shall provide betteroutcomes in these patients

10202016 unusual giomas 51

10202016 52unusual giomas

Content supportndash youmann neurosurgery textbook

Management amp Outcome

bull Quite rare tumorsbull Surgical excision appears to

be best favoured modalitybull Actuarial survival trates are

91 at 5 years and 82 at10 years

bull Median survival 18 years

bull Total resection should beattempted in every case asfar as possible

bull Resection of cyst lining isnot indicated

bull Data regarding the use ofadjuvant therapy at thistime are limited to few casereports only

bull Pts who had incompleteresection high mitoticindex necrosis should bereserved for strict vigilanceand CEMRI on follow ups

10202016 30unusual giomas

bull At progression repeatresection be attempted

bull Proression should betaken as aggressivetumor type

bull Malignanttransformation maynecessitate adjuvanttherapy

10202016 31unusual giomas

bull Br J Neurosurg 2013 Dec27(6)759-64 doi 103109026886972013776666 Epub 2013 Mar 20

bull Pleomorphic xanthoastrocytoma long-term results of surgical treatment and analysis of prognosticfactors

bull Gallo P1 Cecchi PC Locatelli F Rizzo P Ghimenton C Gerosa M Pinna G

bull BACKGROUND

bull Pleomorphic Xanthoastrocytoma (PXA) is a rare brain tumour most commonly affecting children andyoung adults To date only few data regarding the long-term follow-up of these patients after surgery are

available The aim of this study is to describe our single-institution experience in the surgicalmanagement of this particular glioma over a period of over 18 years

bull METHODS

bull We performed a retrospective review of all cases of PXA (40 patients) operated upon at theDepartment of Neurosurgery of Verona Italy between 1990 and 2008 The impact of clinical radiologicalsurgical and histological factors on overall survival (OS) and progression-free survival (PFS) was analysed bymeans of univariate and multivariate models

bull FINDINGS

bull We achieved a gross total resection (GTR) in 65 of patients Histological diagnosis was of grade II in 80anaplastic features were present in the remaining 20 Adjuvant treatment radiotherapy or chemo-

radiotherapy was administered in 40 of the cases Median follow-up was 74 months OS at 5- and10 years was 7632 and 6824 respectively PFS at 5- and 10 years was 71 and 58respectively In the multivariate model histological grade extent of resection and age at diagnosis (le 30years vs gt 30 years) were the only independent prognostic factors for both OS and PFS

bull CONCLUSIONS

bull Our retrospective long-term study confirms the relatively favourable prognosis associatedwith PXA Young patients with a low-grade tumour (WHO grade II) who underwent GTR carrythe longest OS and PFS

10202016 32unusual giomas

bull Brain Tumor Pathol 2006 Apr23(1)55-63

bull A case of anaplastic pleomorphic xanthoastrocytoma presenting with tumor bleeding and cerebrospinal fluid dissemination

bull Asano K1 Miyamoto S Kubo O Kikkukawa T Yagihashi A Ohkuma H

bull Pleomorphic xanthoastrocytoma (PXA) has been considered an astrocytic tumorwith a relatively favorable prognosis However PXA cases having several recurrent patterns with poor prognosis have been reported in recent years and a new concept of anaplastic PXA has been proposed The present case was a 59-year-old woman who presented with tumor bleeding onset and cerebrospinal fluid dissemination The patient had sudden-onset right hemiparesis aphasia and consciousness disturbance and was admitted to a local area hospital After emergency surgery had removed the hematoma postoperative contrast-enhanced CT scan revealed a left temporal tumor A second surgery was therefore performed for initial tumor removal 2 months later Histopathological findings showed that the tumor was typical PXA with strong pleomorphism and xanthomatous changes and contained an ependymoma-like component in the center area However endothelial proliferation and mitosis were more remarkable compared to ordinary PXA The MIB-1 labeling index was 98 high From these findings the histopathological diagnosis was anaplastic PXA The patient underwent surgery to remove recurrent tumors 5 and 16 months later The patient died 36 months after the first onset and CT revealed glioblastoma-like

findings and cerebrospinal fluid dissemination This case report is the first case in which PXA presented with tumor bleeding onset Histopathological findings suggested anaplastic PXA from the first surgical specimens and PXA recurred many times We thus believe that the patient displayed primary anaplastic PXA rather than secondary anaplastic PXA that results in malignant transformation10202016 33unusual giomas

Ganglioglioma

bull WHO grade 1bull Initially described by

Perkins in 1926bull Benign well

differentiatedneuroepithelial tumors

bull Most commonly found intemporal lobe though canoccur anywhere in CNS

bull Commonly found inyoung patients withepilepsy

bull Most commonly notedtumor in temporal lobeepilepsy patients

bull Have been described inbrain steem spinal cordand cerebellum

bull Account for 1 ofprimary brain tumors

10202016 34unusual giomas

bull Male dominencebull Most common features

are seizures headachedizziness ataxiaprogressive weakness

bull Malignanttransformation is rare

bull Histology showsimmature mixture ofabnormal neural andglial elements

bull Macroscopically appearsolid or cystic

bull Cystic are welldelineatedfromsurrounding brain

bull Calcification is common

10202016 35unusual giomas

bull IHC staing shows reactivity for GFAP S-100 andvimentin

bull Neurons show synaptophysin class III betatubulin NFP chromogranin reactivity

10202016 36unusual giomas

Imaging

bull Highly variable imagingcharacters

bull No special typicalimaging criteria

bull Enhancement patternsare highly variable

bull Iso to hypointense onT1WI

bull Heterogenous andhyperintense on T2WI

bull Can contain solid cyticcalcified components

10202016 37unusual giomas

bull FDG PET scan showshypometabolism oreumetabolism in thesepatients

10202016 38unusual giomas

Management And Outcome

bull Gold standard is grosstotal resection

bull Seizure control aftersurgery is as high as80

bull Survival rates are highbull Event free survival of

low grade and highgrade gangiogioma are57 vs 15

bull The optimal surgicalresection depends uponthe eloquent areasinvolved in the vicinityof the tumor

bull The use ofchemotherapy is notwell established

bull Typically they shouldbe allowed for chemoand radiotherapy

10202016 39unusual giomas

bull The use of adjuvant therapy is warranted in thosecases which harbour residual lesions or features ofanaplasia

bull Potential harm to nearby brain areas preclude thejudicious use of radiation

10202016 40unusual giomas

bull Brain Tumor Res Treat 2014 Oct 2(2) 49ndash55

bull Treatment and Outcomes for Gangliogliomas A Single-Center Review of 16 Patients

bull Jye Young Song Jeong Hoon Kim Young Hyun Cho Chang Jin Kim and Eun Jung Lee

bull Ganglioglioma is a rare and slowly growing benign tumor We investigated the outcomes of patients who underwent different combination treatments

bull Methods

bull Between 1998 and 2012 16 patients including 11 men and 5 women with a median age of 125 years (range 25-65 years) were treated for intracranial gangliogliomas at our institution The median follow-up period was 57 years (range 48 days-156 years) Fifteen cases were included in the outcome assessment because one patient was lost to follow-up Complete resection was achieved in 8 (53) patients Six (40) patients underwent incomplete resection with or without adjuvant radiotherapy and one patient with a brainstem tumor underwent only stereotactic biopsy

bull Results

bull Gangliogliomas predominantly affected young (875) male patients and most frequently presented with seizures (64) Of eight patients who underwent complete resection seven did not show recurrence whereas only three of six with incomplete resection showed no recurrence Four patients with recurrence received salvage treatments (two repeat surgeries and two radiosurgeries) A tumor control rate of 93 (1415) was achieved at the last follow-up No recurrence or malignant changes were observed after a median follow-up of 12 and 45 years in four patients who received gamma knife (GK) radiosurgery as adjuvant and salvage treatment

bull Conclusion

bull Complete resection produced the best outcomes and incomplete resection followed by adjuvant or salvage treatments showed favorable outcomes In patients who are not eligible for complete resection because of tumor location or potential neurologic deficits following surgery GK radiosurgery should be considered for the treatment of residual or recurrent tumors

10202016 41unusual giomas

Papillary tumours of pineal

bull WHO grade II AND IIIbull Relatively new edition to WHObull They are equal in both sexesbull Reach medical attention due to headache mental

status changes and altered visionbull Contain eosinophilic cells with distinctive

borders with large pleomorphic nucleibull Positive for cytokeratin synaptophysin stain

variably for GFAP vimentin S-100

10202016 unusual giomas 42

Imaging

bull Generally found to be diffusely enhancinglesion of pineal region

bull Post 3rd ventricle with HCPbull Appear well demarcated from thalamus and

cerebellum

10202016 unusual giomas 43

Mangement

bull Standard treatment is excisionbull Grow slowly and donot transform to

malignant tumorsbull Local recurrence is however possiblebull Radiotherapy appears to provide good control

in local recurrence

10202016 unusual giomas 44

Choroid Glioma Of Third Ventricle

bull WHO Grade IIbull Ist recognised by WHO in

1998bull Arise in the anterior third

ventriclebull Commonly come to

attention after HCPbull Appera to contain in 3rd

ventricle and thought toarise from ventricularsurface

bull Composed of GFAP andvimentin positive cells inmucinous matrix

bull Nuclei are uniform insize and shape

bull Indolent growthbull May show EGF receptor

positivitybull Some suggets these tumor

arise from the lminaterminalis andventricular tanycytes

10202016 45unusual giomas

Imaging

bull Commonly fill 3rd

ventriclebull Diffusely enhance with

contrastbull Appear to infiltrate the

thalamusbull Large tumors may be

difficult to differentiatefrom suprasellar masses

10202016 46unusual giomas

10202016 47unusual giomas

bull Surgical management is limited by locationbull Access through the dialted ventricle and lamina

terminalis may be challangingbull Subtotal resection is thus commonbull Role of adjuvant radiotherapy is unknown

10202016 48unusual giomas

bull Acta Neurochir (Wien) 2008 Nov150(11)1183-91 doi 101007s00701-008-0014-6 Epub

2008 Oct 21

bull Chordoid glioma of the third ventricle

bull Vanhauwaert DJ1 Clement F Van Dorpe J Deruytter MJKGROUND

bull Chordoid glioma is a rare tumour (World Health Organisation grade II) originating from the third ventricle

with both glial and chordoid features It was first described by Brat in 1998 Since there is no detailed

information available on the outcome after surgery and adjuvant treatment we reviewed the literature

bull METHODS

bull A literature search through PUBMED revealed 50 cases of chordoid glioma Most reports

were found in pathology journals Information on the postoperative course was sometimes

very limited We reviewed the available literature and studied in detail the presenting

symptoms mortality and postoperative complications in relation to the extent of resective

surgery as well as the importance of adjuvant treatment

bull CONCLUSIONS

bull Mortality in the immediate postoperative period is 32 and is higher after gross total

resection as compared to subtotal resection Non-fatal postoperative complications are

hypothalamic disorders and mental alterations Gross total resection is the treatment of

choice since no recurrence has been reported after macroscopically complete resection

but this is often difficult because of the location and adherence to the hypothalamus The

role of postoperative radiotherapy is uncertain There is some indication that radiosurgery

with or without conventional irradiation is superior to conventional radiation alone Planned

subtotal resection followed by stereotactic radiosurgery can be a safe and effective

alternative in a patient in whom gross total resection is considered to be too risky

There is no report on the use of chemotherapy in the treatment of chordoid gliomas More

information about the optimal treatment strategy is needed and more reports are also needed10202016 49unusual giomas

bull Neurologist 2011 Jan17(1)52bull Chordoid glioma a case report and literature reviewbull Liu WP1 Cheng JX Yi XC Zhen HN Fei Z Li Q Zhang Xbull chordoid glioma is a rare tumor (World Health Organization grade II) with both

glial and chordoid features often located in the suprasellar region and anterior third ventricle It was first described by Brat in 1998 Because there is no detailed information available from the clinical perspective we reviewed the literature

bull METHODS

bull a literature search through PUBMED and CNKI revealed 64 cases of chordoid glioma Information on the clinical course was very limited We reviewed the literature and studied the pathologic and imaging features postoperative mortality and morbidity in relation to surgical extension and approaches and the importance of adjuvant treatment

bull CONCLUSIONS bull mortality in the immediate postoperative period is 28 and postoperative

morbidity is 60 which are statistically higher after gross total resection as compared with subtotal resection Translamina terminalis approach is considered to be the best approach The current study cannot document that patients have longer survival and higher quality of life after gross total resection than subtotal resection The role of postoperative radiotherapy is uncertain and there is no report on the use of chemotherapy More information about the optimal treatment strategy is needed

10202016 50unusual giomas

Conclusion

bull They are identified by their unique morphologicalfeatures

bull Many of these have indolent course and identified byepilepsy

bull Potentail diagnosis should be considered in youngpatients with seizures and cortically based lesions

bull Correct diagnosis requires experiencedneuropathologisrt

bull As more experienced is gained more knowledgetowards better understanding shall provide betteroutcomes in these patients

10202016 unusual giomas 51

10202016 52unusual giomas

Content supportndash youmann neurosurgery textbook

bull At progression repeatresection be attempted

bull Proression should betaken as aggressivetumor type

bull Malignanttransformation maynecessitate adjuvanttherapy

10202016 31unusual giomas

bull Br J Neurosurg 2013 Dec27(6)759-64 doi 103109026886972013776666 Epub 2013 Mar 20

bull Pleomorphic xanthoastrocytoma long-term results of surgical treatment and analysis of prognosticfactors

bull Gallo P1 Cecchi PC Locatelli F Rizzo P Ghimenton C Gerosa M Pinna G

bull BACKGROUND

bull Pleomorphic Xanthoastrocytoma (PXA) is a rare brain tumour most commonly affecting children andyoung adults To date only few data regarding the long-term follow-up of these patients after surgery are

available The aim of this study is to describe our single-institution experience in the surgicalmanagement of this particular glioma over a period of over 18 years

bull METHODS

bull We performed a retrospective review of all cases of PXA (40 patients) operated upon at theDepartment of Neurosurgery of Verona Italy between 1990 and 2008 The impact of clinical radiologicalsurgical and histological factors on overall survival (OS) and progression-free survival (PFS) was analysed bymeans of univariate and multivariate models

bull FINDINGS

bull We achieved a gross total resection (GTR) in 65 of patients Histological diagnosis was of grade II in 80anaplastic features were present in the remaining 20 Adjuvant treatment radiotherapy or chemo-

radiotherapy was administered in 40 of the cases Median follow-up was 74 months OS at 5- and10 years was 7632 and 6824 respectively PFS at 5- and 10 years was 71 and 58respectively In the multivariate model histological grade extent of resection and age at diagnosis (le 30years vs gt 30 years) were the only independent prognostic factors for both OS and PFS

bull CONCLUSIONS

bull Our retrospective long-term study confirms the relatively favourable prognosis associatedwith PXA Young patients with a low-grade tumour (WHO grade II) who underwent GTR carrythe longest OS and PFS

10202016 32unusual giomas

bull Brain Tumor Pathol 2006 Apr23(1)55-63

bull A case of anaplastic pleomorphic xanthoastrocytoma presenting with tumor bleeding and cerebrospinal fluid dissemination

bull Asano K1 Miyamoto S Kubo O Kikkukawa T Yagihashi A Ohkuma H

bull Pleomorphic xanthoastrocytoma (PXA) has been considered an astrocytic tumorwith a relatively favorable prognosis However PXA cases having several recurrent patterns with poor prognosis have been reported in recent years and a new concept of anaplastic PXA has been proposed The present case was a 59-year-old woman who presented with tumor bleeding onset and cerebrospinal fluid dissemination The patient had sudden-onset right hemiparesis aphasia and consciousness disturbance and was admitted to a local area hospital After emergency surgery had removed the hematoma postoperative contrast-enhanced CT scan revealed a left temporal tumor A second surgery was therefore performed for initial tumor removal 2 months later Histopathological findings showed that the tumor was typical PXA with strong pleomorphism and xanthomatous changes and contained an ependymoma-like component in the center area However endothelial proliferation and mitosis were more remarkable compared to ordinary PXA The MIB-1 labeling index was 98 high From these findings the histopathological diagnosis was anaplastic PXA The patient underwent surgery to remove recurrent tumors 5 and 16 months later The patient died 36 months after the first onset and CT revealed glioblastoma-like

findings and cerebrospinal fluid dissemination This case report is the first case in which PXA presented with tumor bleeding onset Histopathological findings suggested anaplastic PXA from the first surgical specimens and PXA recurred many times We thus believe that the patient displayed primary anaplastic PXA rather than secondary anaplastic PXA that results in malignant transformation10202016 33unusual giomas

Ganglioglioma

bull WHO grade 1bull Initially described by

Perkins in 1926bull Benign well

differentiatedneuroepithelial tumors

bull Most commonly found intemporal lobe though canoccur anywhere in CNS

bull Commonly found inyoung patients withepilepsy

bull Most commonly notedtumor in temporal lobeepilepsy patients

bull Have been described inbrain steem spinal cordand cerebellum

bull Account for 1 ofprimary brain tumors

10202016 34unusual giomas

bull Male dominencebull Most common features

are seizures headachedizziness ataxiaprogressive weakness

bull Malignanttransformation is rare

bull Histology showsimmature mixture ofabnormal neural andglial elements

bull Macroscopically appearsolid or cystic

bull Cystic are welldelineatedfromsurrounding brain

bull Calcification is common

10202016 35unusual giomas

bull IHC staing shows reactivity for GFAP S-100 andvimentin

bull Neurons show synaptophysin class III betatubulin NFP chromogranin reactivity

10202016 36unusual giomas

Imaging

bull Highly variable imagingcharacters

bull No special typicalimaging criteria

bull Enhancement patternsare highly variable

bull Iso to hypointense onT1WI

bull Heterogenous andhyperintense on T2WI

bull Can contain solid cyticcalcified components

10202016 37unusual giomas

bull FDG PET scan showshypometabolism oreumetabolism in thesepatients

10202016 38unusual giomas

Management And Outcome

bull Gold standard is grosstotal resection

bull Seizure control aftersurgery is as high as80

bull Survival rates are highbull Event free survival of

low grade and highgrade gangiogioma are57 vs 15

bull The optimal surgicalresection depends uponthe eloquent areasinvolved in the vicinityof the tumor

bull The use ofchemotherapy is notwell established

bull Typically they shouldbe allowed for chemoand radiotherapy

10202016 39unusual giomas

bull The use of adjuvant therapy is warranted in thosecases which harbour residual lesions or features ofanaplasia

bull Potential harm to nearby brain areas preclude thejudicious use of radiation

10202016 40unusual giomas

bull Brain Tumor Res Treat 2014 Oct 2(2) 49ndash55

bull Treatment and Outcomes for Gangliogliomas A Single-Center Review of 16 Patients

bull Jye Young Song Jeong Hoon Kim Young Hyun Cho Chang Jin Kim and Eun Jung Lee

bull Ganglioglioma is a rare and slowly growing benign tumor We investigated the outcomes of patients who underwent different combination treatments

bull Methods

bull Between 1998 and 2012 16 patients including 11 men and 5 women with a median age of 125 years (range 25-65 years) were treated for intracranial gangliogliomas at our institution The median follow-up period was 57 years (range 48 days-156 years) Fifteen cases were included in the outcome assessment because one patient was lost to follow-up Complete resection was achieved in 8 (53) patients Six (40) patients underwent incomplete resection with or without adjuvant radiotherapy and one patient with a brainstem tumor underwent only stereotactic biopsy

bull Results

bull Gangliogliomas predominantly affected young (875) male patients and most frequently presented with seizures (64) Of eight patients who underwent complete resection seven did not show recurrence whereas only three of six with incomplete resection showed no recurrence Four patients with recurrence received salvage treatments (two repeat surgeries and two radiosurgeries) A tumor control rate of 93 (1415) was achieved at the last follow-up No recurrence or malignant changes were observed after a median follow-up of 12 and 45 years in four patients who received gamma knife (GK) radiosurgery as adjuvant and salvage treatment

bull Conclusion

bull Complete resection produced the best outcomes and incomplete resection followed by adjuvant or salvage treatments showed favorable outcomes In patients who are not eligible for complete resection because of tumor location or potential neurologic deficits following surgery GK radiosurgery should be considered for the treatment of residual or recurrent tumors

10202016 41unusual giomas

Papillary tumours of pineal

bull WHO grade II AND IIIbull Relatively new edition to WHObull They are equal in both sexesbull Reach medical attention due to headache mental

status changes and altered visionbull Contain eosinophilic cells with distinctive

borders with large pleomorphic nucleibull Positive for cytokeratin synaptophysin stain

variably for GFAP vimentin S-100

10202016 unusual giomas 42

Imaging

bull Generally found to be diffusely enhancinglesion of pineal region

bull Post 3rd ventricle with HCPbull Appear well demarcated from thalamus and

cerebellum

10202016 unusual giomas 43

Mangement

bull Standard treatment is excisionbull Grow slowly and donot transform to

malignant tumorsbull Local recurrence is however possiblebull Radiotherapy appears to provide good control

in local recurrence

10202016 unusual giomas 44

Choroid Glioma Of Third Ventricle

bull WHO Grade IIbull Ist recognised by WHO in

1998bull Arise in the anterior third

ventriclebull Commonly come to

attention after HCPbull Appera to contain in 3rd

ventricle and thought toarise from ventricularsurface

bull Composed of GFAP andvimentin positive cells inmucinous matrix

bull Nuclei are uniform insize and shape

bull Indolent growthbull May show EGF receptor

positivitybull Some suggets these tumor

arise from the lminaterminalis andventricular tanycytes

10202016 45unusual giomas

Imaging

bull Commonly fill 3rd

ventriclebull Diffusely enhance with

contrastbull Appear to infiltrate the

thalamusbull Large tumors may be

difficult to differentiatefrom suprasellar masses

10202016 46unusual giomas

10202016 47unusual giomas

bull Surgical management is limited by locationbull Access through the dialted ventricle and lamina

terminalis may be challangingbull Subtotal resection is thus commonbull Role of adjuvant radiotherapy is unknown

10202016 48unusual giomas

bull Acta Neurochir (Wien) 2008 Nov150(11)1183-91 doi 101007s00701-008-0014-6 Epub

2008 Oct 21

bull Chordoid glioma of the third ventricle

bull Vanhauwaert DJ1 Clement F Van Dorpe J Deruytter MJKGROUND

bull Chordoid glioma is a rare tumour (World Health Organisation grade II) originating from the third ventricle

with both glial and chordoid features It was first described by Brat in 1998 Since there is no detailed

information available on the outcome after surgery and adjuvant treatment we reviewed the literature

bull METHODS

bull A literature search through PUBMED revealed 50 cases of chordoid glioma Most reports

were found in pathology journals Information on the postoperative course was sometimes

very limited We reviewed the available literature and studied in detail the presenting

symptoms mortality and postoperative complications in relation to the extent of resective

surgery as well as the importance of adjuvant treatment

bull CONCLUSIONS

bull Mortality in the immediate postoperative period is 32 and is higher after gross total

resection as compared to subtotal resection Non-fatal postoperative complications are

hypothalamic disorders and mental alterations Gross total resection is the treatment of

choice since no recurrence has been reported after macroscopically complete resection

but this is often difficult because of the location and adherence to the hypothalamus The

role of postoperative radiotherapy is uncertain There is some indication that radiosurgery

with or without conventional irradiation is superior to conventional radiation alone Planned

subtotal resection followed by stereotactic radiosurgery can be a safe and effective

alternative in a patient in whom gross total resection is considered to be too risky

There is no report on the use of chemotherapy in the treatment of chordoid gliomas More

information about the optimal treatment strategy is needed and more reports are also needed10202016 49unusual giomas

bull Neurologist 2011 Jan17(1)52bull Chordoid glioma a case report and literature reviewbull Liu WP1 Cheng JX Yi XC Zhen HN Fei Z Li Q Zhang Xbull chordoid glioma is a rare tumor (World Health Organization grade II) with both

glial and chordoid features often located in the suprasellar region and anterior third ventricle It was first described by Brat in 1998 Because there is no detailed information available from the clinical perspective we reviewed the literature

bull METHODS

bull a literature search through PUBMED and CNKI revealed 64 cases of chordoid glioma Information on the clinical course was very limited We reviewed the literature and studied the pathologic and imaging features postoperative mortality and morbidity in relation to surgical extension and approaches and the importance of adjuvant treatment

bull CONCLUSIONS bull mortality in the immediate postoperative period is 28 and postoperative

morbidity is 60 which are statistically higher after gross total resection as compared with subtotal resection Translamina terminalis approach is considered to be the best approach The current study cannot document that patients have longer survival and higher quality of life after gross total resection than subtotal resection The role of postoperative radiotherapy is uncertain and there is no report on the use of chemotherapy More information about the optimal treatment strategy is needed

10202016 50unusual giomas

Conclusion

bull They are identified by their unique morphologicalfeatures

bull Many of these have indolent course and identified byepilepsy

bull Potentail diagnosis should be considered in youngpatients with seizures and cortically based lesions

bull Correct diagnosis requires experiencedneuropathologisrt

bull As more experienced is gained more knowledgetowards better understanding shall provide betteroutcomes in these patients

10202016 unusual giomas 51

10202016 52unusual giomas

Content supportndash youmann neurosurgery textbook

bull Br J Neurosurg 2013 Dec27(6)759-64 doi 103109026886972013776666 Epub 2013 Mar 20

bull Pleomorphic xanthoastrocytoma long-term results of surgical treatment and analysis of prognosticfactors

bull Gallo P1 Cecchi PC Locatelli F Rizzo P Ghimenton C Gerosa M Pinna G

bull BACKGROUND

bull Pleomorphic Xanthoastrocytoma (PXA) is a rare brain tumour most commonly affecting children andyoung adults To date only few data regarding the long-term follow-up of these patients after surgery are

available The aim of this study is to describe our single-institution experience in the surgicalmanagement of this particular glioma over a period of over 18 years

bull METHODS

bull We performed a retrospective review of all cases of PXA (40 patients) operated upon at theDepartment of Neurosurgery of Verona Italy between 1990 and 2008 The impact of clinical radiologicalsurgical and histological factors on overall survival (OS) and progression-free survival (PFS) was analysed bymeans of univariate and multivariate models

bull FINDINGS

bull We achieved a gross total resection (GTR) in 65 of patients Histological diagnosis was of grade II in 80anaplastic features were present in the remaining 20 Adjuvant treatment radiotherapy or chemo-

radiotherapy was administered in 40 of the cases Median follow-up was 74 months OS at 5- and10 years was 7632 and 6824 respectively PFS at 5- and 10 years was 71 and 58respectively In the multivariate model histological grade extent of resection and age at diagnosis (le 30years vs gt 30 years) were the only independent prognostic factors for both OS and PFS

bull CONCLUSIONS

bull Our retrospective long-term study confirms the relatively favourable prognosis associatedwith PXA Young patients with a low-grade tumour (WHO grade II) who underwent GTR carrythe longest OS and PFS

10202016 32unusual giomas

bull Brain Tumor Pathol 2006 Apr23(1)55-63

bull A case of anaplastic pleomorphic xanthoastrocytoma presenting with tumor bleeding and cerebrospinal fluid dissemination

bull Asano K1 Miyamoto S Kubo O Kikkukawa T Yagihashi A Ohkuma H

bull Pleomorphic xanthoastrocytoma (PXA) has been considered an astrocytic tumorwith a relatively favorable prognosis However PXA cases having several recurrent patterns with poor prognosis have been reported in recent years and a new concept of anaplastic PXA has been proposed The present case was a 59-year-old woman who presented with tumor bleeding onset and cerebrospinal fluid dissemination The patient had sudden-onset right hemiparesis aphasia and consciousness disturbance and was admitted to a local area hospital After emergency surgery had removed the hematoma postoperative contrast-enhanced CT scan revealed a left temporal tumor A second surgery was therefore performed for initial tumor removal 2 months later Histopathological findings showed that the tumor was typical PXA with strong pleomorphism and xanthomatous changes and contained an ependymoma-like component in the center area However endothelial proliferation and mitosis were more remarkable compared to ordinary PXA The MIB-1 labeling index was 98 high From these findings the histopathological diagnosis was anaplastic PXA The patient underwent surgery to remove recurrent tumors 5 and 16 months later The patient died 36 months after the first onset and CT revealed glioblastoma-like

findings and cerebrospinal fluid dissemination This case report is the first case in which PXA presented with tumor bleeding onset Histopathological findings suggested anaplastic PXA from the first surgical specimens and PXA recurred many times We thus believe that the patient displayed primary anaplastic PXA rather than secondary anaplastic PXA that results in malignant transformation10202016 33unusual giomas

Ganglioglioma

bull WHO grade 1bull Initially described by

Perkins in 1926bull Benign well

differentiatedneuroepithelial tumors

bull Most commonly found intemporal lobe though canoccur anywhere in CNS

bull Commonly found inyoung patients withepilepsy

bull Most commonly notedtumor in temporal lobeepilepsy patients

bull Have been described inbrain steem spinal cordand cerebellum

bull Account for 1 ofprimary brain tumors

10202016 34unusual giomas

bull Male dominencebull Most common features

are seizures headachedizziness ataxiaprogressive weakness

bull Malignanttransformation is rare

bull Histology showsimmature mixture ofabnormal neural andglial elements

bull Macroscopically appearsolid or cystic

bull Cystic are welldelineatedfromsurrounding brain

bull Calcification is common

10202016 35unusual giomas

bull IHC staing shows reactivity for GFAP S-100 andvimentin

bull Neurons show synaptophysin class III betatubulin NFP chromogranin reactivity

10202016 36unusual giomas

Imaging

bull Highly variable imagingcharacters

bull No special typicalimaging criteria

bull Enhancement patternsare highly variable

bull Iso to hypointense onT1WI

bull Heterogenous andhyperintense on T2WI

bull Can contain solid cyticcalcified components

10202016 37unusual giomas

bull FDG PET scan showshypometabolism oreumetabolism in thesepatients

10202016 38unusual giomas

Management And Outcome

bull Gold standard is grosstotal resection

bull Seizure control aftersurgery is as high as80

bull Survival rates are highbull Event free survival of

low grade and highgrade gangiogioma are57 vs 15

bull The optimal surgicalresection depends uponthe eloquent areasinvolved in the vicinityof the tumor

bull The use ofchemotherapy is notwell established

bull Typically they shouldbe allowed for chemoand radiotherapy

10202016 39unusual giomas

bull The use of adjuvant therapy is warranted in thosecases which harbour residual lesions or features ofanaplasia

bull Potential harm to nearby brain areas preclude thejudicious use of radiation

10202016 40unusual giomas

bull Brain Tumor Res Treat 2014 Oct 2(2) 49ndash55

bull Treatment and Outcomes for Gangliogliomas A Single-Center Review of 16 Patients

bull Jye Young Song Jeong Hoon Kim Young Hyun Cho Chang Jin Kim and Eun Jung Lee

bull Ganglioglioma is a rare and slowly growing benign tumor We investigated the outcomes of patients who underwent different combination treatments

bull Methods

bull Between 1998 and 2012 16 patients including 11 men and 5 women with a median age of 125 years (range 25-65 years) were treated for intracranial gangliogliomas at our institution The median follow-up period was 57 years (range 48 days-156 years) Fifteen cases were included in the outcome assessment because one patient was lost to follow-up Complete resection was achieved in 8 (53) patients Six (40) patients underwent incomplete resection with or without adjuvant radiotherapy and one patient with a brainstem tumor underwent only stereotactic biopsy

bull Results

bull Gangliogliomas predominantly affected young (875) male patients and most frequently presented with seizures (64) Of eight patients who underwent complete resection seven did not show recurrence whereas only three of six with incomplete resection showed no recurrence Four patients with recurrence received salvage treatments (two repeat surgeries and two radiosurgeries) A tumor control rate of 93 (1415) was achieved at the last follow-up No recurrence or malignant changes were observed after a median follow-up of 12 and 45 years in four patients who received gamma knife (GK) radiosurgery as adjuvant and salvage treatment

bull Conclusion

bull Complete resection produced the best outcomes and incomplete resection followed by adjuvant or salvage treatments showed favorable outcomes In patients who are not eligible for complete resection because of tumor location or potential neurologic deficits following surgery GK radiosurgery should be considered for the treatment of residual or recurrent tumors

10202016 41unusual giomas

Papillary tumours of pineal

bull WHO grade II AND IIIbull Relatively new edition to WHObull They are equal in both sexesbull Reach medical attention due to headache mental

status changes and altered visionbull Contain eosinophilic cells with distinctive

borders with large pleomorphic nucleibull Positive for cytokeratin synaptophysin stain

variably for GFAP vimentin S-100

10202016 unusual giomas 42

Imaging

bull Generally found to be diffusely enhancinglesion of pineal region

bull Post 3rd ventricle with HCPbull Appear well demarcated from thalamus and

cerebellum

10202016 unusual giomas 43

Mangement

bull Standard treatment is excisionbull Grow slowly and donot transform to

malignant tumorsbull Local recurrence is however possiblebull Radiotherapy appears to provide good control

in local recurrence

10202016 unusual giomas 44

Choroid Glioma Of Third Ventricle

bull WHO Grade IIbull Ist recognised by WHO in

1998bull Arise in the anterior third

ventriclebull Commonly come to

attention after HCPbull Appera to contain in 3rd

ventricle and thought toarise from ventricularsurface

bull Composed of GFAP andvimentin positive cells inmucinous matrix

bull Nuclei are uniform insize and shape

bull Indolent growthbull May show EGF receptor

positivitybull Some suggets these tumor

arise from the lminaterminalis andventricular tanycytes

10202016 45unusual giomas

Imaging

bull Commonly fill 3rd

ventriclebull Diffusely enhance with

contrastbull Appear to infiltrate the

thalamusbull Large tumors may be

difficult to differentiatefrom suprasellar masses

10202016 46unusual giomas

10202016 47unusual giomas

bull Surgical management is limited by locationbull Access through the dialted ventricle and lamina

terminalis may be challangingbull Subtotal resection is thus commonbull Role of adjuvant radiotherapy is unknown

10202016 48unusual giomas

bull Acta Neurochir (Wien) 2008 Nov150(11)1183-91 doi 101007s00701-008-0014-6 Epub

2008 Oct 21

bull Chordoid glioma of the third ventricle

bull Vanhauwaert DJ1 Clement F Van Dorpe J Deruytter MJKGROUND

bull Chordoid glioma is a rare tumour (World Health Organisation grade II) originating from the third ventricle

with both glial and chordoid features It was first described by Brat in 1998 Since there is no detailed

information available on the outcome after surgery and adjuvant treatment we reviewed the literature

bull METHODS

bull A literature search through PUBMED revealed 50 cases of chordoid glioma Most reports

were found in pathology journals Information on the postoperative course was sometimes

very limited We reviewed the available literature and studied in detail the presenting

symptoms mortality and postoperative complications in relation to the extent of resective

surgery as well as the importance of adjuvant treatment

bull CONCLUSIONS

bull Mortality in the immediate postoperative period is 32 and is higher after gross total

resection as compared to subtotal resection Non-fatal postoperative complications are

hypothalamic disorders and mental alterations Gross total resection is the treatment of

choice since no recurrence has been reported after macroscopically complete resection

but this is often difficult because of the location and adherence to the hypothalamus The

role of postoperative radiotherapy is uncertain There is some indication that radiosurgery

with or without conventional irradiation is superior to conventional radiation alone Planned

subtotal resection followed by stereotactic radiosurgery can be a safe and effective

alternative in a patient in whom gross total resection is considered to be too risky

There is no report on the use of chemotherapy in the treatment of chordoid gliomas More

information about the optimal treatment strategy is needed and more reports are also needed10202016 49unusual giomas

bull Neurologist 2011 Jan17(1)52bull Chordoid glioma a case report and literature reviewbull Liu WP1 Cheng JX Yi XC Zhen HN Fei Z Li Q Zhang Xbull chordoid glioma is a rare tumor (World Health Organization grade II) with both

glial and chordoid features often located in the suprasellar region and anterior third ventricle It was first described by Brat in 1998 Because there is no detailed information available from the clinical perspective we reviewed the literature

bull METHODS

bull a literature search through PUBMED and CNKI revealed 64 cases of chordoid glioma Information on the clinical course was very limited We reviewed the literature and studied the pathologic and imaging features postoperative mortality and morbidity in relation to surgical extension and approaches and the importance of adjuvant treatment

bull CONCLUSIONS bull mortality in the immediate postoperative period is 28 and postoperative

morbidity is 60 which are statistically higher after gross total resection as compared with subtotal resection Translamina terminalis approach is considered to be the best approach The current study cannot document that patients have longer survival and higher quality of life after gross total resection than subtotal resection The role of postoperative radiotherapy is uncertain and there is no report on the use of chemotherapy More information about the optimal treatment strategy is needed

10202016 50unusual giomas

Conclusion

bull They are identified by their unique morphologicalfeatures

bull Many of these have indolent course and identified byepilepsy

bull Potentail diagnosis should be considered in youngpatients with seizures and cortically based lesions

bull Correct diagnosis requires experiencedneuropathologisrt

bull As more experienced is gained more knowledgetowards better understanding shall provide betteroutcomes in these patients

10202016 unusual giomas 51

10202016 52unusual giomas

Content supportndash youmann neurosurgery textbook

bull Brain Tumor Pathol 2006 Apr23(1)55-63

bull A case of anaplastic pleomorphic xanthoastrocytoma presenting with tumor bleeding and cerebrospinal fluid dissemination

bull Asano K1 Miyamoto S Kubo O Kikkukawa T Yagihashi A Ohkuma H

bull Pleomorphic xanthoastrocytoma (PXA) has been considered an astrocytic tumorwith a relatively favorable prognosis However PXA cases having several recurrent patterns with poor prognosis have been reported in recent years and a new concept of anaplastic PXA has been proposed The present case was a 59-year-old woman who presented with tumor bleeding onset and cerebrospinal fluid dissemination The patient had sudden-onset right hemiparesis aphasia and consciousness disturbance and was admitted to a local area hospital After emergency surgery had removed the hematoma postoperative contrast-enhanced CT scan revealed a left temporal tumor A second surgery was therefore performed for initial tumor removal 2 months later Histopathological findings showed that the tumor was typical PXA with strong pleomorphism and xanthomatous changes and contained an ependymoma-like component in the center area However endothelial proliferation and mitosis were more remarkable compared to ordinary PXA The MIB-1 labeling index was 98 high From these findings the histopathological diagnosis was anaplastic PXA The patient underwent surgery to remove recurrent tumors 5 and 16 months later The patient died 36 months after the first onset and CT revealed glioblastoma-like

findings and cerebrospinal fluid dissemination This case report is the first case in which PXA presented with tumor bleeding onset Histopathological findings suggested anaplastic PXA from the first surgical specimens and PXA recurred many times We thus believe that the patient displayed primary anaplastic PXA rather than secondary anaplastic PXA that results in malignant transformation10202016 33unusual giomas

Ganglioglioma

bull WHO grade 1bull Initially described by

Perkins in 1926bull Benign well

differentiatedneuroepithelial tumors

bull Most commonly found intemporal lobe though canoccur anywhere in CNS

bull Commonly found inyoung patients withepilepsy

bull Most commonly notedtumor in temporal lobeepilepsy patients

bull Have been described inbrain steem spinal cordand cerebellum

bull Account for 1 ofprimary brain tumors

10202016 34unusual giomas

bull Male dominencebull Most common features

are seizures headachedizziness ataxiaprogressive weakness

bull Malignanttransformation is rare

bull Histology showsimmature mixture ofabnormal neural andglial elements

bull Macroscopically appearsolid or cystic

bull Cystic are welldelineatedfromsurrounding brain

bull Calcification is common

10202016 35unusual giomas

bull IHC staing shows reactivity for GFAP S-100 andvimentin

bull Neurons show synaptophysin class III betatubulin NFP chromogranin reactivity

10202016 36unusual giomas

Imaging

bull Highly variable imagingcharacters

bull No special typicalimaging criteria

bull Enhancement patternsare highly variable

bull Iso to hypointense onT1WI

bull Heterogenous andhyperintense on T2WI

bull Can contain solid cyticcalcified components

10202016 37unusual giomas

bull FDG PET scan showshypometabolism oreumetabolism in thesepatients

10202016 38unusual giomas

Management And Outcome

bull Gold standard is grosstotal resection

bull Seizure control aftersurgery is as high as80

bull Survival rates are highbull Event free survival of

low grade and highgrade gangiogioma are57 vs 15

bull The optimal surgicalresection depends uponthe eloquent areasinvolved in the vicinityof the tumor

bull The use ofchemotherapy is notwell established

bull Typically they shouldbe allowed for chemoand radiotherapy

10202016 39unusual giomas

bull The use of adjuvant therapy is warranted in thosecases which harbour residual lesions or features ofanaplasia

bull Potential harm to nearby brain areas preclude thejudicious use of radiation

10202016 40unusual giomas

bull Brain Tumor Res Treat 2014 Oct 2(2) 49ndash55

bull Treatment and Outcomes for Gangliogliomas A Single-Center Review of 16 Patients

bull Jye Young Song Jeong Hoon Kim Young Hyun Cho Chang Jin Kim and Eun Jung Lee

bull Ganglioglioma is a rare and slowly growing benign tumor We investigated the outcomes of patients who underwent different combination treatments

bull Methods

bull Between 1998 and 2012 16 patients including 11 men and 5 women with a median age of 125 years (range 25-65 years) were treated for intracranial gangliogliomas at our institution The median follow-up period was 57 years (range 48 days-156 years) Fifteen cases were included in the outcome assessment because one patient was lost to follow-up Complete resection was achieved in 8 (53) patients Six (40) patients underwent incomplete resection with or without adjuvant radiotherapy and one patient with a brainstem tumor underwent only stereotactic biopsy

bull Results

bull Gangliogliomas predominantly affected young (875) male patients and most frequently presented with seizures (64) Of eight patients who underwent complete resection seven did not show recurrence whereas only three of six with incomplete resection showed no recurrence Four patients with recurrence received salvage treatments (two repeat surgeries and two radiosurgeries) A tumor control rate of 93 (1415) was achieved at the last follow-up No recurrence or malignant changes were observed after a median follow-up of 12 and 45 years in four patients who received gamma knife (GK) radiosurgery as adjuvant and salvage treatment

bull Conclusion

bull Complete resection produced the best outcomes and incomplete resection followed by adjuvant or salvage treatments showed favorable outcomes In patients who are not eligible for complete resection because of tumor location or potential neurologic deficits following surgery GK radiosurgery should be considered for the treatment of residual or recurrent tumors

10202016 41unusual giomas

Papillary tumours of pineal

bull WHO grade II AND IIIbull Relatively new edition to WHObull They are equal in both sexesbull Reach medical attention due to headache mental

status changes and altered visionbull Contain eosinophilic cells with distinctive

borders with large pleomorphic nucleibull Positive for cytokeratin synaptophysin stain

variably for GFAP vimentin S-100

10202016 unusual giomas 42

Imaging

bull Generally found to be diffusely enhancinglesion of pineal region

bull Post 3rd ventricle with HCPbull Appear well demarcated from thalamus and

cerebellum

10202016 unusual giomas 43

Mangement

bull Standard treatment is excisionbull Grow slowly and donot transform to

malignant tumorsbull Local recurrence is however possiblebull Radiotherapy appears to provide good control

in local recurrence

10202016 unusual giomas 44

Choroid Glioma Of Third Ventricle

bull WHO Grade IIbull Ist recognised by WHO in

1998bull Arise in the anterior third

ventriclebull Commonly come to

attention after HCPbull Appera to contain in 3rd

ventricle and thought toarise from ventricularsurface

bull Composed of GFAP andvimentin positive cells inmucinous matrix

bull Nuclei are uniform insize and shape

bull Indolent growthbull May show EGF receptor

positivitybull Some suggets these tumor

arise from the lminaterminalis andventricular tanycytes

10202016 45unusual giomas

Imaging

bull Commonly fill 3rd

ventriclebull Diffusely enhance with

contrastbull Appear to infiltrate the

thalamusbull Large tumors may be

difficult to differentiatefrom suprasellar masses

10202016 46unusual giomas

10202016 47unusual giomas

bull Surgical management is limited by locationbull Access through the dialted ventricle and lamina

terminalis may be challangingbull Subtotal resection is thus commonbull Role of adjuvant radiotherapy is unknown

10202016 48unusual giomas

bull Acta Neurochir (Wien) 2008 Nov150(11)1183-91 doi 101007s00701-008-0014-6 Epub

2008 Oct 21

bull Chordoid glioma of the third ventricle

bull Vanhauwaert DJ1 Clement F Van Dorpe J Deruytter MJKGROUND

bull Chordoid glioma is a rare tumour (World Health Organisation grade II) originating from the third ventricle

with both glial and chordoid features It was first described by Brat in 1998 Since there is no detailed

information available on the outcome after surgery and adjuvant treatment we reviewed the literature

bull METHODS

bull A literature search through PUBMED revealed 50 cases of chordoid glioma Most reports

were found in pathology journals Information on the postoperative course was sometimes

very limited We reviewed the available literature and studied in detail the presenting

symptoms mortality and postoperative complications in relation to the extent of resective

surgery as well as the importance of adjuvant treatment

bull CONCLUSIONS

bull Mortality in the immediate postoperative period is 32 and is higher after gross total

resection as compared to subtotal resection Non-fatal postoperative complications are

hypothalamic disorders and mental alterations Gross total resection is the treatment of

choice since no recurrence has been reported after macroscopically complete resection

but this is often difficult because of the location and adherence to the hypothalamus The

role of postoperative radiotherapy is uncertain There is some indication that radiosurgery

with or without conventional irradiation is superior to conventional radiation alone Planned

subtotal resection followed by stereotactic radiosurgery can be a safe and effective

alternative in a patient in whom gross total resection is considered to be too risky

There is no report on the use of chemotherapy in the treatment of chordoid gliomas More

information about the optimal treatment strategy is needed and more reports are also needed10202016 49unusual giomas

bull Neurologist 2011 Jan17(1)52bull Chordoid glioma a case report and literature reviewbull Liu WP1 Cheng JX Yi XC Zhen HN Fei Z Li Q Zhang Xbull chordoid glioma is a rare tumor (World Health Organization grade II) with both

glial and chordoid features often located in the suprasellar region and anterior third ventricle It was first described by Brat in 1998 Because there is no detailed information available from the clinical perspective we reviewed the literature

bull METHODS

bull a literature search through PUBMED and CNKI revealed 64 cases of chordoid glioma Information on the clinical course was very limited We reviewed the literature and studied the pathologic and imaging features postoperative mortality and morbidity in relation to surgical extension and approaches and the importance of adjuvant treatment

bull CONCLUSIONS bull mortality in the immediate postoperative period is 28 and postoperative

morbidity is 60 which are statistically higher after gross total resection as compared with subtotal resection Translamina terminalis approach is considered to be the best approach The current study cannot document that patients have longer survival and higher quality of life after gross total resection than subtotal resection The role of postoperative radiotherapy is uncertain and there is no report on the use of chemotherapy More information about the optimal treatment strategy is needed

10202016 50unusual giomas

Conclusion

bull They are identified by their unique morphologicalfeatures

bull Many of these have indolent course and identified byepilepsy

bull Potentail diagnosis should be considered in youngpatients with seizures and cortically based lesions

bull Correct diagnosis requires experiencedneuropathologisrt

bull As more experienced is gained more knowledgetowards better understanding shall provide betteroutcomes in these patients

10202016 unusual giomas 51

10202016 52unusual giomas

Content supportndash youmann neurosurgery textbook

Ganglioglioma

bull WHO grade 1bull Initially described by

Perkins in 1926bull Benign well

differentiatedneuroepithelial tumors

bull Most commonly found intemporal lobe though canoccur anywhere in CNS

bull Commonly found inyoung patients withepilepsy

bull Most commonly notedtumor in temporal lobeepilepsy patients

bull Have been described inbrain steem spinal cordand cerebellum

bull Account for 1 ofprimary brain tumors

10202016 34unusual giomas

bull Male dominencebull Most common features

are seizures headachedizziness ataxiaprogressive weakness

bull Malignanttransformation is rare

bull Histology showsimmature mixture ofabnormal neural andglial elements

bull Macroscopically appearsolid or cystic

bull Cystic are welldelineatedfromsurrounding brain

bull Calcification is common

10202016 35unusual giomas

bull IHC staing shows reactivity for GFAP S-100 andvimentin

bull Neurons show synaptophysin class III betatubulin NFP chromogranin reactivity

10202016 36unusual giomas

Imaging

bull Highly variable imagingcharacters

bull No special typicalimaging criteria

bull Enhancement patternsare highly variable

bull Iso to hypointense onT1WI

bull Heterogenous andhyperintense on T2WI

bull Can contain solid cyticcalcified components

10202016 37unusual giomas

bull FDG PET scan showshypometabolism oreumetabolism in thesepatients

10202016 38unusual giomas

Management And Outcome

bull Gold standard is grosstotal resection

bull Seizure control aftersurgery is as high as80

bull Survival rates are highbull Event free survival of

low grade and highgrade gangiogioma are57 vs 15

bull The optimal surgicalresection depends uponthe eloquent areasinvolved in the vicinityof the tumor

bull The use ofchemotherapy is notwell established

bull Typically they shouldbe allowed for chemoand radiotherapy

10202016 39unusual giomas

bull The use of adjuvant therapy is warranted in thosecases which harbour residual lesions or features ofanaplasia

bull Potential harm to nearby brain areas preclude thejudicious use of radiation

10202016 40unusual giomas

bull Brain Tumor Res Treat 2014 Oct 2(2) 49ndash55

bull Treatment and Outcomes for Gangliogliomas A Single-Center Review of 16 Patients

bull Jye Young Song Jeong Hoon Kim Young Hyun Cho Chang Jin Kim and Eun Jung Lee

bull Ganglioglioma is a rare and slowly growing benign tumor We investigated the outcomes of patients who underwent different combination treatments

bull Methods

bull Between 1998 and 2012 16 patients including 11 men and 5 women with a median age of 125 years (range 25-65 years) were treated for intracranial gangliogliomas at our institution The median follow-up period was 57 years (range 48 days-156 years) Fifteen cases were included in the outcome assessment because one patient was lost to follow-up Complete resection was achieved in 8 (53) patients Six (40) patients underwent incomplete resection with or without adjuvant radiotherapy and one patient with a brainstem tumor underwent only stereotactic biopsy

bull Results

bull Gangliogliomas predominantly affected young (875) male patients and most frequently presented with seizures (64) Of eight patients who underwent complete resection seven did not show recurrence whereas only three of six with incomplete resection showed no recurrence Four patients with recurrence received salvage treatments (two repeat surgeries and two radiosurgeries) A tumor control rate of 93 (1415) was achieved at the last follow-up No recurrence or malignant changes were observed after a median follow-up of 12 and 45 years in four patients who received gamma knife (GK) radiosurgery as adjuvant and salvage treatment

bull Conclusion

bull Complete resection produced the best outcomes and incomplete resection followed by adjuvant or salvage treatments showed favorable outcomes In patients who are not eligible for complete resection because of tumor location or potential neurologic deficits following surgery GK radiosurgery should be considered for the treatment of residual or recurrent tumors

10202016 41unusual giomas

Papillary tumours of pineal

bull WHO grade II AND IIIbull Relatively new edition to WHObull They are equal in both sexesbull Reach medical attention due to headache mental

status changes and altered visionbull Contain eosinophilic cells with distinctive

borders with large pleomorphic nucleibull Positive for cytokeratin synaptophysin stain

variably for GFAP vimentin S-100

10202016 unusual giomas 42

Imaging

bull Generally found to be diffusely enhancinglesion of pineal region

bull Post 3rd ventricle with HCPbull Appear well demarcated from thalamus and

cerebellum

10202016 unusual giomas 43

Mangement

bull Standard treatment is excisionbull Grow slowly and donot transform to

malignant tumorsbull Local recurrence is however possiblebull Radiotherapy appears to provide good control

in local recurrence

10202016 unusual giomas 44

Choroid Glioma Of Third Ventricle

bull WHO Grade IIbull Ist recognised by WHO in

1998bull Arise in the anterior third

ventriclebull Commonly come to

attention after HCPbull Appera to contain in 3rd

ventricle and thought toarise from ventricularsurface

bull Composed of GFAP andvimentin positive cells inmucinous matrix

bull Nuclei are uniform insize and shape

bull Indolent growthbull May show EGF receptor

positivitybull Some suggets these tumor

arise from the lminaterminalis andventricular tanycytes

10202016 45unusual giomas

Imaging

bull Commonly fill 3rd

ventriclebull Diffusely enhance with

contrastbull Appear to infiltrate the

thalamusbull Large tumors may be

difficult to differentiatefrom suprasellar masses

10202016 46unusual giomas

10202016 47unusual giomas

bull Surgical management is limited by locationbull Access through the dialted ventricle and lamina

terminalis may be challangingbull Subtotal resection is thus commonbull Role of adjuvant radiotherapy is unknown

10202016 48unusual giomas

bull Acta Neurochir (Wien) 2008 Nov150(11)1183-91 doi 101007s00701-008-0014-6 Epub

2008 Oct 21

bull Chordoid glioma of the third ventricle

bull Vanhauwaert DJ1 Clement F Van Dorpe J Deruytter MJKGROUND

bull Chordoid glioma is a rare tumour (World Health Organisation grade II) originating from the third ventricle

with both glial and chordoid features It was first described by Brat in 1998 Since there is no detailed

information available on the outcome after surgery and adjuvant treatment we reviewed the literature

bull METHODS

bull A literature search through PUBMED revealed 50 cases of chordoid glioma Most reports

were found in pathology journals Information on the postoperative course was sometimes

very limited We reviewed the available literature and studied in detail the presenting

symptoms mortality and postoperative complications in relation to the extent of resective

surgery as well as the importance of adjuvant treatment

bull CONCLUSIONS

bull Mortality in the immediate postoperative period is 32 and is higher after gross total

resection as compared to subtotal resection Non-fatal postoperative complications are

hypothalamic disorders and mental alterations Gross total resection is the treatment of

choice since no recurrence has been reported after macroscopically complete resection

but this is often difficult because of the location and adherence to the hypothalamus The

role of postoperative radiotherapy is uncertain There is some indication that radiosurgery

with or without conventional irradiation is superior to conventional radiation alone Planned

subtotal resection followed by stereotactic radiosurgery can be a safe and effective

alternative in a patient in whom gross total resection is considered to be too risky

There is no report on the use of chemotherapy in the treatment of chordoid gliomas More

information about the optimal treatment strategy is needed and more reports are also needed10202016 49unusual giomas

bull Neurologist 2011 Jan17(1)52bull Chordoid glioma a case report and literature reviewbull Liu WP1 Cheng JX Yi XC Zhen HN Fei Z Li Q Zhang Xbull chordoid glioma is a rare tumor (World Health Organization grade II) with both

glial and chordoid features often located in the suprasellar region and anterior third ventricle It was first described by Brat in 1998 Because there is no detailed information available from the clinical perspective we reviewed the literature

bull METHODS

bull a literature search through PUBMED and CNKI revealed 64 cases of chordoid glioma Information on the clinical course was very limited We reviewed the literature and studied the pathologic and imaging features postoperative mortality and morbidity in relation to surgical extension and approaches and the importance of adjuvant treatment

bull CONCLUSIONS bull mortality in the immediate postoperative period is 28 and postoperative

morbidity is 60 which are statistically higher after gross total resection as compared with subtotal resection Translamina terminalis approach is considered to be the best approach The current study cannot document that patients have longer survival and higher quality of life after gross total resection than subtotal resection The role of postoperative radiotherapy is uncertain and there is no report on the use of chemotherapy More information about the optimal treatment strategy is needed

10202016 50unusual giomas

Conclusion

bull They are identified by their unique morphologicalfeatures

bull Many of these have indolent course and identified byepilepsy

bull Potentail diagnosis should be considered in youngpatients with seizures and cortically based lesions

bull Correct diagnosis requires experiencedneuropathologisrt

bull As more experienced is gained more knowledgetowards better understanding shall provide betteroutcomes in these patients

10202016 unusual giomas 51

10202016 52unusual giomas

Content supportndash youmann neurosurgery textbook

bull Male dominencebull Most common features

are seizures headachedizziness ataxiaprogressive weakness

bull Malignanttransformation is rare

bull Histology showsimmature mixture ofabnormal neural andglial elements

bull Macroscopically appearsolid or cystic

bull Cystic are welldelineatedfromsurrounding brain

bull Calcification is common

10202016 35unusual giomas

bull IHC staing shows reactivity for GFAP S-100 andvimentin

bull Neurons show synaptophysin class III betatubulin NFP chromogranin reactivity

10202016 36unusual giomas

Imaging

bull Highly variable imagingcharacters

bull No special typicalimaging criteria

bull Enhancement patternsare highly variable

bull Iso to hypointense onT1WI

bull Heterogenous andhyperintense on T2WI

bull Can contain solid cyticcalcified components

10202016 37unusual giomas

bull FDG PET scan showshypometabolism oreumetabolism in thesepatients

10202016 38unusual giomas

Management And Outcome

bull Gold standard is grosstotal resection

bull Seizure control aftersurgery is as high as80

bull Survival rates are highbull Event free survival of

low grade and highgrade gangiogioma are57 vs 15

bull The optimal surgicalresection depends uponthe eloquent areasinvolved in the vicinityof the tumor

bull The use ofchemotherapy is notwell established

bull Typically they shouldbe allowed for chemoand radiotherapy

10202016 39unusual giomas

bull The use of adjuvant therapy is warranted in thosecases which harbour residual lesions or features ofanaplasia

bull Potential harm to nearby brain areas preclude thejudicious use of radiation

10202016 40unusual giomas

bull Brain Tumor Res Treat 2014 Oct 2(2) 49ndash55

bull Treatment and Outcomes for Gangliogliomas A Single-Center Review of 16 Patients

bull Jye Young Song Jeong Hoon Kim Young Hyun Cho Chang Jin Kim and Eun Jung Lee

bull Ganglioglioma is a rare and slowly growing benign tumor We investigated the outcomes of patients who underwent different combination treatments

bull Methods

bull Between 1998 and 2012 16 patients including 11 men and 5 women with a median age of 125 years (range 25-65 years) were treated for intracranial gangliogliomas at our institution The median follow-up period was 57 years (range 48 days-156 years) Fifteen cases were included in the outcome assessment because one patient was lost to follow-up Complete resection was achieved in 8 (53) patients Six (40) patients underwent incomplete resection with or without adjuvant radiotherapy and one patient with a brainstem tumor underwent only stereotactic biopsy

bull Results

bull Gangliogliomas predominantly affected young (875) male patients and most frequently presented with seizures (64) Of eight patients who underwent complete resection seven did not show recurrence whereas only three of six with incomplete resection showed no recurrence Four patients with recurrence received salvage treatments (two repeat surgeries and two radiosurgeries) A tumor control rate of 93 (1415) was achieved at the last follow-up No recurrence or malignant changes were observed after a median follow-up of 12 and 45 years in four patients who received gamma knife (GK) radiosurgery as adjuvant and salvage treatment

bull Conclusion

bull Complete resection produced the best outcomes and incomplete resection followed by adjuvant or salvage treatments showed favorable outcomes In patients who are not eligible for complete resection because of tumor location or potential neurologic deficits following surgery GK radiosurgery should be considered for the treatment of residual or recurrent tumors

10202016 41unusual giomas

Papillary tumours of pineal

bull WHO grade II AND IIIbull Relatively new edition to WHObull They are equal in both sexesbull Reach medical attention due to headache mental

status changes and altered visionbull Contain eosinophilic cells with distinctive

borders with large pleomorphic nucleibull Positive for cytokeratin synaptophysin stain

variably for GFAP vimentin S-100

10202016 unusual giomas 42

Imaging

bull Generally found to be diffusely enhancinglesion of pineal region

bull Post 3rd ventricle with HCPbull Appear well demarcated from thalamus and

cerebellum

10202016 unusual giomas 43

Mangement

bull Standard treatment is excisionbull Grow slowly and donot transform to

malignant tumorsbull Local recurrence is however possiblebull Radiotherapy appears to provide good control

in local recurrence

10202016 unusual giomas 44

Choroid Glioma Of Third Ventricle

bull WHO Grade IIbull Ist recognised by WHO in

1998bull Arise in the anterior third

ventriclebull Commonly come to

attention after HCPbull Appera to contain in 3rd

ventricle and thought toarise from ventricularsurface

bull Composed of GFAP andvimentin positive cells inmucinous matrix

bull Nuclei are uniform insize and shape

bull Indolent growthbull May show EGF receptor

positivitybull Some suggets these tumor

arise from the lminaterminalis andventricular tanycytes

10202016 45unusual giomas

Imaging

bull Commonly fill 3rd

ventriclebull Diffusely enhance with

contrastbull Appear to infiltrate the

thalamusbull Large tumors may be

difficult to differentiatefrom suprasellar masses

10202016 46unusual giomas

10202016 47unusual giomas

bull Surgical management is limited by locationbull Access through the dialted ventricle and lamina

terminalis may be challangingbull Subtotal resection is thus commonbull Role of adjuvant radiotherapy is unknown

10202016 48unusual giomas

bull Acta Neurochir (Wien) 2008 Nov150(11)1183-91 doi 101007s00701-008-0014-6 Epub

2008 Oct 21

bull Chordoid glioma of the third ventricle

bull Vanhauwaert DJ1 Clement F Van Dorpe J Deruytter MJKGROUND

bull Chordoid glioma is a rare tumour (World Health Organisation grade II) originating from the third ventricle

with both glial and chordoid features It was first described by Brat in 1998 Since there is no detailed

information available on the outcome after surgery and adjuvant treatment we reviewed the literature

bull METHODS

bull A literature search through PUBMED revealed 50 cases of chordoid glioma Most reports

were found in pathology journals Information on the postoperative course was sometimes

very limited We reviewed the available literature and studied in detail the presenting

symptoms mortality and postoperative complications in relation to the extent of resective

surgery as well as the importance of adjuvant treatment

bull CONCLUSIONS

bull Mortality in the immediate postoperative period is 32 and is higher after gross total

resection as compared to subtotal resection Non-fatal postoperative complications are

hypothalamic disorders and mental alterations Gross total resection is the treatment of

choice since no recurrence has been reported after macroscopically complete resection

but this is often difficult because of the location and adherence to the hypothalamus The

role of postoperative radiotherapy is uncertain There is some indication that radiosurgery

with or without conventional irradiation is superior to conventional radiation alone Planned

subtotal resection followed by stereotactic radiosurgery can be a safe and effective

alternative in a patient in whom gross total resection is considered to be too risky

There is no report on the use of chemotherapy in the treatment of chordoid gliomas More

information about the optimal treatment strategy is needed and more reports are also needed10202016 49unusual giomas

bull Neurologist 2011 Jan17(1)52bull Chordoid glioma a case report and literature reviewbull Liu WP1 Cheng JX Yi XC Zhen HN Fei Z Li Q Zhang Xbull chordoid glioma is a rare tumor (World Health Organization grade II) with both

glial and chordoid features often located in the suprasellar region and anterior third ventricle It was first described by Brat in 1998 Because there is no detailed information available from the clinical perspective we reviewed the literature

bull METHODS

bull a literature search through PUBMED and CNKI revealed 64 cases of chordoid glioma Information on the clinical course was very limited We reviewed the literature and studied the pathologic and imaging features postoperative mortality and morbidity in relation to surgical extension and approaches and the importance of adjuvant treatment

bull CONCLUSIONS bull mortality in the immediate postoperative period is 28 and postoperative

morbidity is 60 which are statistically higher after gross total resection as compared with subtotal resection Translamina terminalis approach is considered to be the best approach The current study cannot document that patients have longer survival and higher quality of life after gross total resection than subtotal resection The role of postoperative radiotherapy is uncertain and there is no report on the use of chemotherapy More information about the optimal treatment strategy is needed

10202016 50unusual giomas

Conclusion

bull They are identified by their unique morphologicalfeatures

bull Many of these have indolent course and identified byepilepsy

bull Potentail diagnosis should be considered in youngpatients with seizures and cortically based lesions

bull Correct diagnosis requires experiencedneuropathologisrt

bull As more experienced is gained more knowledgetowards better understanding shall provide betteroutcomes in these patients

10202016 unusual giomas 51

10202016 52unusual giomas

Content supportndash youmann neurosurgery textbook

bull IHC staing shows reactivity for GFAP S-100 andvimentin

bull Neurons show synaptophysin class III betatubulin NFP chromogranin reactivity

10202016 36unusual giomas

Imaging

bull Highly variable imagingcharacters

bull No special typicalimaging criteria

bull Enhancement patternsare highly variable

bull Iso to hypointense onT1WI

bull Heterogenous andhyperintense on T2WI

bull Can contain solid cyticcalcified components

10202016 37unusual giomas

bull FDG PET scan showshypometabolism oreumetabolism in thesepatients

10202016 38unusual giomas

Management And Outcome

bull Gold standard is grosstotal resection

bull Seizure control aftersurgery is as high as80

bull Survival rates are highbull Event free survival of

low grade and highgrade gangiogioma are57 vs 15

bull The optimal surgicalresection depends uponthe eloquent areasinvolved in the vicinityof the tumor

bull The use ofchemotherapy is notwell established

bull Typically they shouldbe allowed for chemoand radiotherapy

10202016 39unusual giomas

bull The use of adjuvant therapy is warranted in thosecases which harbour residual lesions or features ofanaplasia

bull Potential harm to nearby brain areas preclude thejudicious use of radiation

10202016 40unusual giomas

bull Brain Tumor Res Treat 2014 Oct 2(2) 49ndash55

bull Treatment and Outcomes for Gangliogliomas A Single-Center Review of 16 Patients

bull Jye Young Song Jeong Hoon Kim Young Hyun Cho Chang Jin Kim and Eun Jung Lee

bull Ganglioglioma is a rare and slowly growing benign tumor We investigated the outcomes of patients who underwent different combination treatments

bull Methods

bull Between 1998 and 2012 16 patients including 11 men and 5 women with a median age of 125 years (range 25-65 years) were treated for intracranial gangliogliomas at our institution The median follow-up period was 57 years (range 48 days-156 years) Fifteen cases were included in the outcome assessment because one patient was lost to follow-up Complete resection was achieved in 8 (53) patients Six (40) patients underwent incomplete resection with or without adjuvant radiotherapy and one patient with a brainstem tumor underwent only stereotactic biopsy

bull Results

bull Gangliogliomas predominantly affected young (875) male patients and most frequently presented with seizures (64) Of eight patients who underwent complete resection seven did not show recurrence whereas only three of six with incomplete resection showed no recurrence Four patients with recurrence received salvage treatments (two repeat surgeries and two radiosurgeries) A tumor control rate of 93 (1415) was achieved at the last follow-up No recurrence or malignant changes were observed after a median follow-up of 12 and 45 years in four patients who received gamma knife (GK) radiosurgery as adjuvant and salvage treatment

bull Conclusion

bull Complete resection produced the best outcomes and incomplete resection followed by adjuvant or salvage treatments showed favorable outcomes In patients who are not eligible for complete resection because of tumor location or potential neurologic deficits following surgery GK radiosurgery should be considered for the treatment of residual or recurrent tumors

10202016 41unusual giomas

Papillary tumours of pineal

bull WHO grade II AND IIIbull Relatively new edition to WHObull They are equal in both sexesbull Reach medical attention due to headache mental

status changes and altered visionbull Contain eosinophilic cells with distinctive

borders with large pleomorphic nucleibull Positive for cytokeratin synaptophysin stain

variably for GFAP vimentin S-100

10202016 unusual giomas 42

Imaging

bull Generally found to be diffusely enhancinglesion of pineal region

bull Post 3rd ventricle with HCPbull Appear well demarcated from thalamus and

cerebellum

10202016 unusual giomas 43

Mangement

bull Standard treatment is excisionbull Grow slowly and donot transform to

malignant tumorsbull Local recurrence is however possiblebull Radiotherapy appears to provide good control

in local recurrence

10202016 unusual giomas 44

Choroid Glioma Of Third Ventricle

bull WHO Grade IIbull Ist recognised by WHO in

1998bull Arise in the anterior third

ventriclebull Commonly come to

attention after HCPbull Appera to contain in 3rd

ventricle and thought toarise from ventricularsurface

bull Composed of GFAP andvimentin positive cells inmucinous matrix

bull Nuclei are uniform insize and shape

bull Indolent growthbull May show EGF receptor

positivitybull Some suggets these tumor

arise from the lminaterminalis andventricular tanycytes

10202016 45unusual giomas

Imaging

bull Commonly fill 3rd

ventriclebull Diffusely enhance with

contrastbull Appear to infiltrate the

thalamusbull Large tumors may be

difficult to differentiatefrom suprasellar masses

10202016 46unusual giomas

10202016 47unusual giomas

bull Surgical management is limited by locationbull Access through the dialted ventricle and lamina

terminalis may be challangingbull Subtotal resection is thus commonbull Role of adjuvant radiotherapy is unknown

10202016 48unusual giomas

bull Acta Neurochir (Wien) 2008 Nov150(11)1183-91 doi 101007s00701-008-0014-6 Epub

2008 Oct 21

bull Chordoid glioma of the third ventricle

bull Vanhauwaert DJ1 Clement F Van Dorpe J Deruytter MJKGROUND

bull Chordoid glioma is a rare tumour (World Health Organisation grade II) originating from the third ventricle

with both glial and chordoid features It was first described by Brat in 1998 Since there is no detailed

information available on the outcome after surgery and adjuvant treatment we reviewed the literature

bull METHODS

bull A literature search through PUBMED revealed 50 cases of chordoid glioma Most reports

were found in pathology journals Information on the postoperative course was sometimes

very limited We reviewed the available literature and studied in detail the presenting

symptoms mortality and postoperative complications in relation to the extent of resective

surgery as well as the importance of adjuvant treatment

bull CONCLUSIONS

bull Mortality in the immediate postoperative period is 32 and is higher after gross total

resection as compared to subtotal resection Non-fatal postoperative complications are

hypothalamic disorders and mental alterations Gross total resection is the treatment of

choice since no recurrence has been reported after macroscopically complete resection

but this is often difficult because of the location and adherence to the hypothalamus The

role of postoperative radiotherapy is uncertain There is some indication that radiosurgery

with or without conventional irradiation is superior to conventional radiation alone Planned

subtotal resection followed by stereotactic radiosurgery can be a safe and effective

alternative in a patient in whom gross total resection is considered to be too risky

There is no report on the use of chemotherapy in the treatment of chordoid gliomas More

information about the optimal treatment strategy is needed and more reports are also needed10202016 49unusual giomas

bull Neurologist 2011 Jan17(1)52bull Chordoid glioma a case report and literature reviewbull Liu WP1 Cheng JX Yi XC Zhen HN Fei Z Li Q Zhang Xbull chordoid glioma is a rare tumor (World Health Organization grade II) with both

glial and chordoid features often located in the suprasellar region and anterior third ventricle It was first described by Brat in 1998 Because there is no detailed information available from the clinical perspective we reviewed the literature

bull METHODS

bull a literature search through PUBMED and CNKI revealed 64 cases of chordoid glioma Information on the clinical course was very limited We reviewed the literature and studied the pathologic and imaging features postoperative mortality and morbidity in relation to surgical extension and approaches and the importance of adjuvant treatment

bull CONCLUSIONS bull mortality in the immediate postoperative period is 28 and postoperative

morbidity is 60 which are statistically higher after gross total resection as compared with subtotal resection Translamina terminalis approach is considered to be the best approach The current study cannot document that patients have longer survival and higher quality of life after gross total resection than subtotal resection The role of postoperative radiotherapy is uncertain and there is no report on the use of chemotherapy More information about the optimal treatment strategy is needed

10202016 50unusual giomas

Conclusion

bull They are identified by their unique morphologicalfeatures

bull Many of these have indolent course and identified byepilepsy

bull Potentail diagnosis should be considered in youngpatients with seizures and cortically based lesions

bull Correct diagnosis requires experiencedneuropathologisrt

bull As more experienced is gained more knowledgetowards better understanding shall provide betteroutcomes in these patients

10202016 unusual giomas 51

10202016 52unusual giomas

Content supportndash youmann neurosurgery textbook

Imaging

bull Highly variable imagingcharacters

bull No special typicalimaging criteria

bull Enhancement patternsare highly variable

bull Iso to hypointense onT1WI

bull Heterogenous andhyperintense on T2WI

bull Can contain solid cyticcalcified components

10202016 37unusual giomas

bull FDG PET scan showshypometabolism oreumetabolism in thesepatients

10202016 38unusual giomas

Management And Outcome

bull Gold standard is grosstotal resection

bull Seizure control aftersurgery is as high as80

bull Survival rates are highbull Event free survival of

low grade and highgrade gangiogioma are57 vs 15

bull The optimal surgicalresection depends uponthe eloquent areasinvolved in the vicinityof the tumor

bull The use ofchemotherapy is notwell established

bull Typically they shouldbe allowed for chemoand radiotherapy

10202016 39unusual giomas

bull The use of adjuvant therapy is warranted in thosecases which harbour residual lesions or features ofanaplasia

bull Potential harm to nearby brain areas preclude thejudicious use of radiation

10202016 40unusual giomas

bull Brain Tumor Res Treat 2014 Oct 2(2) 49ndash55

bull Treatment and Outcomes for Gangliogliomas A Single-Center Review of 16 Patients

bull Jye Young Song Jeong Hoon Kim Young Hyun Cho Chang Jin Kim and Eun Jung Lee

bull Ganglioglioma is a rare and slowly growing benign tumor We investigated the outcomes of patients who underwent different combination treatments

bull Methods

bull Between 1998 and 2012 16 patients including 11 men and 5 women with a median age of 125 years (range 25-65 years) were treated for intracranial gangliogliomas at our institution The median follow-up period was 57 years (range 48 days-156 years) Fifteen cases were included in the outcome assessment because one patient was lost to follow-up Complete resection was achieved in 8 (53) patients Six (40) patients underwent incomplete resection with or without adjuvant radiotherapy and one patient with a brainstem tumor underwent only stereotactic biopsy

bull Results

bull Gangliogliomas predominantly affected young (875) male patients and most frequently presented with seizures (64) Of eight patients who underwent complete resection seven did not show recurrence whereas only three of six with incomplete resection showed no recurrence Four patients with recurrence received salvage treatments (two repeat surgeries and two radiosurgeries) A tumor control rate of 93 (1415) was achieved at the last follow-up No recurrence or malignant changes were observed after a median follow-up of 12 and 45 years in four patients who received gamma knife (GK) radiosurgery as adjuvant and salvage treatment

bull Conclusion

bull Complete resection produced the best outcomes and incomplete resection followed by adjuvant or salvage treatments showed favorable outcomes In patients who are not eligible for complete resection because of tumor location or potential neurologic deficits following surgery GK radiosurgery should be considered for the treatment of residual or recurrent tumors

10202016 41unusual giomas

Papillary tumours of pineal

bull WHO grade II AND IIIbull Relatively new edition to WHObull They are equal in both sexesbull Reach medical attention due to headache mental

status changes and altered visionbull Contain eosinophilic cells with distinctive

borders with large pleomorphic nucleibull Positive for cytokeratin synaptophysin stain

variably for GFAP vimentin S-100

10202016 unusual giomas 42

Imaging

bull Generally found to be diffusely enhancinglesion of pineal region

bull Post 3rd ventricle with HCPbull Appear well demarcated from thalamus and

cerebellum

10202016 unusual giomas 43

Mangement

bull Standard treatment is excisionbull Grow slowly and donot transform to

malignant tumorsbull Local recurrence is however possiblebull Radiotherapy appears to provide good control

in local recurrence

10202016 unusual giomas 44

Choroid Glioma Of Third Ventricle

bull WHO Grade IIbull Ist recognised by WHO in

1998bull Arise in the anterior third

ventriclebull Commonly come to

attention after HCPbull Appera to contain in 3rd

ventricle and thought toarise from ventricularsurface

bull Composed of GFAP andvimentin positive cells inmucinous matrix

bull Nuclei are uniform insize and shape

bull Indolent growthbull May show EGF receptor

positivitybull Some suggets these tumor

arise from the lminaterminalis andventricular tanycytes

10202016 45unusual giomas

Imaging

bull Commonly fill 3rd

ventriclebull Diffusely enhance with

contrastbull Appear to infiltrate the

thalamusbull Large tumors may be

difficult to differentiatefrom suprasellar masses

10202016 46unusual giomas

10202016 47unusual giomas

bull Surgical management is limited by locationbull Access through the dialted ventricle and lamina

terminalis may be challangingbull Subtotal resection is thus commonbull Role of adjuvant radiotherapy is unknown

10202016 48unusual giomas

bull Acta Neurochir (Wien) 2008 Nov150(11)1183-91 doi 101007s00701-008-0014-6 Epub

2008 Oct 21

bull Chordoid glioma of the third ventricle

bull Vanhauwaert DJ1 Clement F Van Dorpe J Deruytter MJKGROUND

bull Chordoid glioma is a rare tumour (World Health Organisation grade II) originating from the third ventricle

with both glial and chordoid features It was first described by Brat in 1998 Since there is no detailed

information available on the outcome after surgery and adjuvant treatment we reviewed the literature

bull METHODS

bull A literature search through PUBMED revealed 50 cases of chordoid glioma Most reports

were found in pathology journals Information on the postoperative course was sometimes

very limited We reviewed the available literature and studied in detail the presenting

symptoms mortality and postoperative complications in relation to the extent of resective

surgery as well as the importance of adjuvant treatment

bull CONCLUSIONS

bull Mortality in the immediate postoperative period is 32 and is higher after gross total

resection as compared to subtotal resection Non-fatal postoperative complications are

hypothalamic disorders and mental alterations Gross total resection is the treatment of

choice since no recurrence has been reported after macroscopically complete resection

but this is often difficult because of the location and adherence to the hypothalamus The

role of postoperative radiotherapy is uncertain There is some indication that radiosurgery

with or without conventional irradiation is superior to conventional radiation alone Planned

subtotal resection followed by stereotactic radiosurgery can be a safe and effective

alternative in a patient in whom gross total resection is considered to be too risky

There is no report on the use of chemotherapy in the treatment of chordoid gliomas More

information about the optimal treatment strategy is needed and more reports are also needed10202016 49unusual giomas

bull Neurologist 2011 Jan17(1)52bull Chordoid glioma a case report and literature reviewbull Liu WP1 Cheng JX Yi XC Zhen HN Fei Z Li Q Zhang Xbull chordoid glioma is a rare tumor (World Health Organization grade II) with both

glial and chordoid features often located in the suprasellar region and anterior third ventricle It was first described by Brat in 1998 Because there is no detailed information available from the clinical perspective we reviewed the literature

bull METHODS

bull a literature search through PUBMED and CNKI revealed 64 cases of chordoid glioma Information on the clinical course was very limited We reviewed the literature and studied the pathologic and imaging features postoperative mortality and morbidity in relation to surgical extension and approaches and the importance of adjuvant treatment

bull CONCLUSIONS bull mortality in the immediate postoperative period is 28 and postoperative

morbidity is 60 which are statistically higher after gross total resection as compared with subtotal resection Translamina terminalis approach is considered to be the best approach The current study cannot document that patients have longer survival and higher quality of life after gross total resection than subtotal resection The role of postoperative radiotherapy is uncertain and there is no report on the use of chemotherapy More information about the optimal treatment strategy is needed

10202016 50unusual giomas

Conclusion

bull They are identified by their unique morphologicalfeatures

bull Many of these have indolent course and identified byepilepsy

bull Potentail diagnosis should be considered in youngpatients with seizures and cortically based lesions

bull Correct diagnosis requires experiencedneuropathologisrt

bull As more experienced is gained more knowledgetowards better understanding shall provide betteroutcomes in these patients

10202016 unusual giomas 51

10202016 52unusual giomas

Content supportndash youmann neurosurgery textbook

bull FDG PET scan showshypometabolism oreumetabolism in thesepatients

10202016 38unusual giomas

Management And Outcome

bull Gold standard is grosstotal resection

bull Seizure control aftersurgery is as high as80

bull Survival rates are highbull Event free survival of

low grade and highgrade gangiogioma are57 vs 15

bull The optimal surgicalresection depends uponthe eloquent areasinvolved in the vicinityof the tumor

bull The use ofchemotherapy is notwell established

bull Typically they shouldbe allowed for chemoand radiotherapy

10202016 39unusual giomas

bull The use of adjuvant therapy is warranted in thosecases which harbour residual lesions or features ofanaplasia

bull Potential harm to nearby brain areas preclude thejudicious use of radiation

10202016 40unusual giomas

bull Brain Tumor Res Treat 2014 Oct 2(2) 49ndash55

bull Treatment and Outcomes for Gangliogliomas A Single-Center Review of 16 Patients

bull Jye Young Song Jeong Hoon Kim Young Hyun Cho Chang Jin Kim and Eun Jung Lee

bull Ganglioglioma is a rare and slowly growing benign tumor We investigated the outcomes of patients who underwent different combination treatments

bull Methods

bull Between 1998 and 2012 16 patients including 11 men and 5 women with a median age of 125 years (range 25-65 years) were treated for intracranial gangliogliomas at our institution The median follow-up period was 57 years (range 48 days-156 years) Fifteen cases were included in the outcome assessment because one patient was lost to follow-up Complete resection was achieved in 8 (53) patients Six (40) patients underwent incomplete resection with or without adjuvant radiotherapy and one patient with a brainstem tumor underwent only stereotactic biopsy

bull Results

bull Gangliogliomas predominantly affected young (875) male patients and most frequently presented with seizures (64) Of eight patients who underwent complete resection seven did not show recurrence whereas only three of six with incomplete resection showed no recurrence Four patients with recurrence received salvage treatments (two repeat surgeries and two radiosurgeries) A tumor control rate of 93 (1415) was achieved at the last follow-up No recurrence or malignant changes were observed after a median follow-up of 12 and 45 years in four patients who received gamma knife (GK) radiosurgery as adjuvant and salvage treatment

bull Conclusion

bull Complete resection produced the best outcomes and incomplete resection followed by adjuvant or salvage treatments showed favorable outcomes In patients who are not eligible for complete resection because of tumor location or potential neurologic deficits following surgery GK radiosurgery should be considered for the treatment of residual or recurrent tumors

10202016 41unusual giomas

Papillary tumours of pineal

bull WHO grade II AND IIIbull Relatively new edition to WHObull They are equal in both sexesbull Reach medical attention due to headache mental

status changes and altered visionbull Contain eosinophilic cells with distinctive

borders with large pleomorphic nucleibull Positive for cytokeratin synaptophysin stain

variably for GFAP vimentin S-100

10202016 unusual giomas 42

Imaging

bull Generally found to be diffusely enhancinglesion of pineal region

bull Post 3rd ventricle with HCPbull Appear well demarcated from thalamus and

cerebellum

10202016 unusual giomas 43

Mangement

bull Standard treatment is excisionbull Grow slowly and donot transform to

malignant tumorsbull Local recurrence is however possiblebull Radiotherapy appears to provide good control

in local recurrence

10202016 unusual giomas 44

Choroid Glioma Of Third Ventricle

bull WHO Grade IIbull Ist recognised by WHO in

1998bull Arise in the anterior third

ventriclebull Commonly come to

attention after HCPbull Appera to contain in 3rd

ventricle and thought toarise from ventricularsurface

bull Composed of GFAP andvimentin positive cells inmucinous matrix

bull Nuclei are uniform insize and shape

bull Indolent growthbull May show EGF receptor

positivitybull Some suggets these tumor

arise from the lminaterminalis andventricular tanycytes

10202016 45unusual giomas

Imaging

bull Commonly fill 3rd

ventriclebull Diffusely enhance with

contrastbull Appear to infiltrate the

thalamusbull Large tumors may be

difficult to differentiatefrom suprasellar masses

10202016 46unusual giomas

10202016 47unusual giomas

bull Surgical management is limited by locationbull Access through the dialted ventricle and lamina

terminalis may be challangingbull Subtotal resection is thus commonbull Role of adjuvant radiotherapy is unknown

10202016 48unusual giomas

bull Acta Neurochir (Wien) 2008 Nov150(11)1183-91 doi 101007s00701-008-0014-6 Epub

2008 Oct 21

bull Chordoid glioma of the third ventricle

bull Vanhauwaert DJ1 Clement F Van Dorpe J Deruytter MJKGROUND

bull Chordoid glioma is a rare tumour (World Health Organisation grade II) originating from the third ventricle

with both glial and chordoid features It was first described by Brat in 1998 Since there is no detailed

information available on the outcome after surgery and adjuvant treatment we reviewed the literature

bull METHODS

bull A literature search through PUBMED revealed 50 cases of chordoid glioma Most reports

were found in pathology journals Information on the postoperative course was sometimes

very limited We reviewed the available literature and studied in detail the presenting

symptoms mortality and postoperative complications in relation to the extent of resective

surgery as well as the importance of adjuvant treatment

bull CONCLUSIONS

bull Mortality in the immediate postoperative period is 32 and is higher after gross total

resection as compared to subtotal resection Non-fatal postoperative complications are

hypothalamic disorders and mental alterations Gross total resection is the treatment of

choice since no recurrence has been reported after macroscopically complete resection

but this is often difficult because of the location and adherence to the hypothalamus The

role of postoperative radiotherapy is uncertain There is some indication that radiosurgery

with or without conventional irradiation is superior to conventional radiation alone Planned

subtotal resection followed by stereotactic radiosurgery can be a safe and effective

alternative in a patient in whom gross total resection is considered to be too risky

There is no report on the use of chemotherapy in the treatment of chordoid gliomas More

information about the optimal treatment strategy is needed and more reports are also needed10202016 49unusual giomas

bull Neurologist 2011 Jan17(1)52bull Chordoid glioma a case report and literature reviewbull Liu WP1 Cheng JX Yi XC Zhen HN Fei Z Li Q Zhang Xbull chordoid glioma is a rare tumor (World Health Organization grade II) with both

glial and chordoid features often located in the suprasellar region and anterior third ventricle It was first described by Brat in 1998 Because there is no detailed information available from the clinical perspective we reviewed the literature

bull METHODS

bull a literature search through PUBMED and CNKI revealed 64 cases of chordoid glioma Information on the clinical course was very limited We reviewed the literature and studied the pathologic and imaging features postoperative mortality and morbidity in relation to surgical extension and approaches and the importance of adjuvant treatment

bull CONCLUSIONS bull mortality in the immediate postoperative period is 28 and postoperative

morbidity is 60 which are statistically higher after gross total resection as compared with subtotal resection Translamina terminalis approach is considered to be the best approach The current study cannot document that patients have longer survival and higher quality of life after gross total resection than subtotal resection The role of postoperative radiotherapy is uncertain and there is no report on the use of chemotherapy More information about the optimal treatment strategy is needed

10202016 50unusual giomas

Conclusion

bull They are identified by their unique morphologicalfeatures

bull Many of these have indolent course and identified byepilepsy

bull Potentail diagnosis should be considered in youngpatients with seizures and cortically based lesions

bull Correct diagnosis requires experiencedneuropathologisrt

bull As more experienced is gained more knowledgetowards better understanding shall provide betteroutcomes in these patients

10202016 unusual giomas 51

10202016 52unusual giomas

Content supportndash youmann neurosurgery textbook

Management And Outcome

bull Gold standard is grosstotal resection

bull Seizure control aftersurgery is as high as80

bull Survival rates are highbull Event free survival of

low grade and highgrade gangiogioma are57 vs 15

bull The optimal surgicalresection depends uponthe eloquent areasinvolved in the vicinityof the tumor

bull The use ofchemotherapy is notwell established

bull Typically they shouldbe allowed for chemoand radiotherapy

10202016 39unusual giomas

bull The use of adjuvant therapy is warranted in thosecases which harbour residual lesions or features ofanaplasia

bull Potential harm to nearby brain areas preclude thejudicious use of radiation

10202016 40unusual giomas

bull Brain Tumor Res Treat 2014 Oct 2(2) 49ndash55

bull Treatment and Outcomes for Gangliogliomas A Single-Center Review of 16 Patients

bull Jye Young Song Jeong Hoon Kim Young Hyun Cho Chang Jin Kim and Eun Jung Lee

bull Ganglioglioma is a rare and slowly growing benign tumor We investigated the outcomes of patients who underwent different combination treatments

bull Methods

bull Between 1998 and 2012 16 patients including 11 men and 5 women with a median age of 125 years (range 25-65 years) were treated for intracranial gangliogliomas at our institution The median follow-up period was 57 years (range 48 days-156 years) Fifteen cases were included in the outcome assessment because one patient was lost to follow-up Complete resection was achieved in 8 (53) patients Six (40) patients underwent incomplete resection with or without adjuvant radiotherapy and one patient with a brainstem tumor underwent only stereotactic biopsy

bull Results

bull Gangliogliomas predominantly affected young (875) male patients and most frequently presented with seizures (64) Of eight patients who underwent complete resection seven did not show recurrence whereas only three of six with incomplete resection showed no recurrence Four patients with recurrence received salvage treatments (two repeat surgeries and two radiosurgeries) A tumor control rate of 93 (1415) was achieved at the last follow-up No recurrence or malignant changes were observed after a median follow-up of 12 and 45 years in four patients who received gamma knife (GK) radiosurgery as adjuvant and salvage treatment

bull Conclusion

bull Complete resection produced the best outcomes and incomplete resection followed by adjuvant or salvage treatments showed favorable outcomes In patients who are not eligible for complete resection because of tumor location or potential neurologic deficits following surgery GK radiosurgery should be considered for the treatment of residual or recurrent tumors

10202016 41unusual giomas

Papillary tumours of pineal

bull WHO grade II AND IIIbull Relatively new edition to WHObull They are equal in both sexesbull Reach medical attention due to headache mental

status changes and altered visionbull Contain eosinophilic cells with distinctive

borders with large pleomorphic nucleibull Positive for cytokeratin synaptophysin stain

variably for GFAP vimentin S-100

10202016 unusual giomas 42

Imaging

bull Generally found to be diffusely enhancinglesion of pineal region

bull Post 3rd ventricle with HCPbull Appear well demarcated from thalamus and

cerebellum

10202016 unusual giomas 43

Mangement

bull Standard treatment is excisionbull Grow slowly and donot transform to

malignant tumorsbull Local recurrence is however possiblebull Radiotherapy appears to provide good control

in local recurrence

10202016 unusual giomas 44

Choroid Glioma Of Third Ventricle

bull WHO Grade IIbull Ist recognised by WHO in

1998bull Arise in the anterior third

ventriclebull Commonly come to

attention after HCPbull Appera to contain in 3rd

ventricle and thought toarise from ventricularsurface

bull Composed of GFAP andvimentin positive cells inmucinous matrix

bull Nuclei are uniform insize and shape

bull Indolent growthbull May show EGF receptor

positivitybull Some suggets these tumor

arise from the lminaterminalis andventricular tanycytes

10202016 45unusual giomas

Imaging

bull Commonly fill 3rd

ventriclebull Diffusely enhance with

contrastbull Appear to infiltrate the

thalamusbull Large tumors may be

difficult to differentiatefrom suprasellar masses

10202016 46unusual giomas

10202016 47unusual giomas

bull Surgical management is limited by locationbull Access through the dialted ventricle and lamina

terminalis may be challangingbull Subtotal resection is thus commonbull Role of adjuvant radiotherapy is unknown

10202016 48unusual giomas

bull Acta Neurochir (Wien) 2008 Nov150(11)1183-91 doi 101007s00701-008-0014-6 Epub

2008 Oct 21

bull Chordoid glioma of the third ventricle

bull Vanhauwaert DJ1 Clement F Van Dorpe J Deruytter MJKGROUND

bull Chordoid glioma is a rare tumour (World Health Organisation grade II) originating from the third ventricle

with both glial and chordoid features It was first described by Brat in 1998 Since there is no detailed

information available on the outcome after surgery and adjuvant treatment we reviewed the literature

bull METHODS

bull A literature search through PUBMED revealed 50 cases of chordoid glioma Most reports

were found in pathology journals Information on the postoperative course was sometimes

very limited We reviewed the available literature and studied in detail the presenting

symptoms mortality and postoperative complications in relation to the extent of resective

surgery as well as the importance of adjuvant treatment

bull CONCLUSIONS

bull Mortality in the immediate postoperative period is 32 and is higher after gross total

resection as compared to subtotal resection Non-fatal postoperative complications are

hypothalamic disorders and mental alterations Gross total resection is the treatment of

choice since no recurrence has been reported after macroscopically complete resection

but this is often difficult because of the location and adherence to the hypothalamus The

role of postoperative radiotherapy is uncertain There is some indication that radiosurgery

with or without conventional irradiation is superior to conventional radiation alone Planned

subtotal resection followed by stereotactic radiosurgery can be a safe and effective

alternative in a patient in whom gross total resection is considered to be too risky

There is no report on the use of chemotherapy in the treatment of chordoid gliomas More

information about the optimal treatment strategy is needed and more reports are also needed10202016 49unusual giomas

bull Neurologist 2011 Jan17(1)52bull Chordoid glioma a case report and literature reviewbull Liu WP1 Cheng JX Yi XC Zhen HN Fei Z Li Q Zhang Xbull chordoid glioma is a rare tumor (World Health Organization grade II) with both

glial and chordoid features often located in the suprasellar region and anterior third ventricle It was first described by Brat in 1998 Because there is no detailed information available from the clinical perspective we reviewed the literature

bull METHODS

bull a literature search through PUBMED and CNKI revealed 64 cases of chordoid glioma Information on the clinical course was very limited We reviewed the literature and studied the pathologic and imaging features postoperative mortality and morbidity in relation to surgical extension and approaches and the importance of adjuvant treatment

bull CONCLUSIONS bull mortality in the immediate postoperative period is 28 and postoperative

morbidity is 60 which are statistically higher after gross total resection as compared with subtotal resection Translamina terminalis approach is considered to be the best approach The current study cannot document that patients have longer survival and higher quality of life after gross total resection than subtotal resection The role of postoperative radiotherapy is uncertain and there is no report on the use of chemotherapy More information about the optimal treatment strategy is needed

10202016 50unusual giomas

Conclusion

bull They are identified by their unique morphologicalfeatures

bull Many of these have indolent course and identified byepilepsy

bull Potentail diagnosis should be considered in youngpatients with seizures and cortically based lesions

bull Correct diagnosis requires experiencedneuropathologisrt

bull As more experienced is gained more knowledgetowards better understanding shall provide betteroutcomes in these patients

10202016 unusual giomas 51

10202016 52unusual giomas

Content supportndash youmann neurosurgery textbook

bull The use of adjuvant therapy is warranted in thosecases which harbour residual lesions or features ofanaplasia

bull Potential harm to nearby brain areas preclude thejudicious use of radiation

10202016 40unusual giomas

bull Brain Tumor Res Treat 2014 Oct 2(2) 49ndash55

bull Treatment and Outcomes for Gangliogliomas A Single-Center Review of 16 Patients

bull Jye Young Song Jeong Hoon Kim Young Hyun Cho Chang Jin Kim and Eun Jung Lee

bull Ganglioglioma is a rare and slowly growing benign tumor We investigated the outcomes of patients who underwent different combination treatments

bull Methods

bull Between 1998 and 2012 16 patients including 11 men and 5 women with a median age of 125 years (range 25-65 years) were treated for intracranial gangliogliomas at our institution The median follow-up period was 57 years (range 48 days-156 years) Fifteen cases were included in the outcome assessment because one patient was lost to follow-up Complete resection was achieved in 8 (53) patients Six (40) patients underwent incomplete resection with or without adjuvant radiotherapy and one patient with a brainstem tumor underwent only stereotactic biopsy

bull Results

bull Gangliogliomas predominantly affected young (875) male patients and most frequently presented with seizures (64) Of eight patients who underwent complete resection seven did not show recurrence whereas only three of six with incomplete resection showed no recurrence Four patients with recurrence received salvage treatments (two repeat surgeries and two radiosurgeries) A tumor control rate of 93 (1415) was achieved at the last follow-up No recurrence or malignant changes were observed after a median follow-up of 12 and 45 years in four patients who received gamma knife (GK) radiosurgery as adjuvant and salvage treatment

bull Conclusion

bull Complete resection produced the best outcomes and incomplete resection followed by adjuvant or salvage treatments showed favorable outcomes In patients who are not eligible for complete resection because of tumor location or potential neurologic deficits following surgery GK radiosurgery should be considered for the treatment of residual or recurrent tumors

10202016 41unusual giomas

Papillary tumours of pineal

bull WHO grade II AND IIIbull Relatively new edition to WHObull They are equal in both sexesbull Reach medical attention due to headache mental

status changes and altered visionbull Contain eosinophilic cells with distinctive

borders with large pleomorphic nucleibull Positive for cytokeratin synaptophysin stain

variably for GFAP vimentin S-100

10202016 unusual giomas 42

Imaging

bull Generally found to be diffusely enhancinglesion of pineal region

bull Post 3rd ventricle with HCPbull Appear well demarcated from thalamus and

cerebellum

10202016 unusual giomas 43

Mangement

bull Standard treatment is excisionbull Grow slowly and donot transform to

malignant tumorsbull Local recurrence is however possiblebull Radiotherapy appears to provide good control

in local recurrence

10202016 unusual giomas 44

Choroid Glioma Of Third Ventricle

bull WHO Grade IIbull Ist recognised by WHO in

1998bull Arise in the anterior third

ventriclebull Commonly come to

attention after HCPbull Appera to contain in 3rd

ventricle and thought toarise from ventricularsurface

bull Composed of GFAP andvimentin positive cells inmucinous matrix

bull Nuclei are uniform insize and shape

bull Indolent growthbull May show EGF receptor

positivitybull Some suggets these tumor

arise from the lminaterminalis andventricular tanycytes

10202016 45unusual giomas

Imaging

bull Commonly fill 3rd

ventriclebull Diffusely enhance with

contrastbull Appear to infiltrate the

thalamusbull Large tumors may be

difficult to differentiatefrom suprasellar masses

10202016 46unusual giomas

10202016 47unusual giomas

bull Surgical management is limited by locationbull Access through the dialted ventricle and lamina

terminalis may be challangingbull Subtotal resection is thus commonbull Role of adjuvant radiotherapy is unknown

10202016 48unusual giomas

bull Acta Neurochir (Wien) 2008 Nov150(11)1183-91 doi 101007s00701-008-0014-6 Epub

2008 Oct 21

bull Chordoid glioma of the third ventricle

bull Vanhauwaert DJ1 Clement F Van Dorpe J Deruytter MJKGROUND

bull Chordoid glioma is a rare tumour (World Health Organisation grade II) originating from the third ventricle

with both glial and chordoid features It was first described by Brat in 1998 Since there is no detailed

information available on the outcome after surgery and adjuvant treatment we reviewed the literature

bull METHODS

bull A literature search through PUBMED revealed 50 cases of chordoid glioma Most reports

were found in pathology journals Information on the postoperative course was sometimes

very limited We reviewed the available literature and studied in detail the presenting

symptoms mortality and postoperative complications in relation to the extent of resective

surgery as well as the importance of adjuvant treatment

bull CONCLUSIONS

bull Mortality in the immediate postoperative period is 32 and is higher after gross total

resection as compared to subtotal resection Non-fatal postoperative complications are

hypothalamic disorders and mental alterations Gross total resection is the treatment of

choice since no recurrence has been reported after macroscopically complete resection

but this is often difficult because of the location and adherence to the hypothalamus The

role of postoperative radiotherapy is uncertain There is some indication that radiosurgery

with or without conventional irradiation is superior to conventional radiation alone Planned

subtotal resection followed by stereotactic radiosurgery can be a safe and effective

alternative in a patient in whom gross total resection is considered to be too risky

There is no report on the use of chemotherapy in the treatment of chordoid gliomas More

information about the optimal treatment strategy is needed and more reports are also needed10202016 49unusual giomas

bull Neurologist 2011 Jan17(1)52bull Chordoid glioma a case report and literature reviewbull Liu WP1 Cheng JX Yi XC Zhen HN Fei Z Li Q Zhang Xbull chordoid glioma is a rare tumor (World Health Organization grade II) with both

glial and chordoid features often located in the suprasellar region and anterior third ventricle It was first described by Brat in 1998 Because there is no detailed information available from the clinical perspective we reviewed the literature

bull METHODS

bull a literature search through PUBMED and CNKI revealed 64 cases of chordoid glioma Information on the clinical course was very limited We reviewed the literature and studied the pathologic and imaging features postoperative mortality and morbidity in relation to surgical extension and approaches and the importance of adjuvant treatment

bull CONCLUSIONS bull mortality in the immediate postoperative period is 28 and postoperative

morbidity is 60 which are statistically higher after gross total resection as compared with subtotal resection Translamina terminalis approach is considered to be the best approach The current study cannot document that patients have longer survival and higher quality of life after gross total resection than subtotal resection The role of postoperative radiotherapy is uncertain and there is no report on the use of chemotherapy More information about the optimal treatment strategy is needed

10202016 50unusual giomas

Conclusion

bull They are identified by their unique morphologicalfeatures

bull Many of these have indolent course and identified byepilepsy

bull Potentail diagnosis should be considered in youngpatients with seizures and cortically based lesions

bull Correct diagnosis requires experiencedneuropathologisrt

bull As more experienced is gained more knowledgetowards better understanding shall provide betteroutcomes in these patients

10202016 unusual giomas 51

10202016 52unusual giomas

Content supportndash youmann neurosurgery textbook

bull Brain Tumor Res Treat 2014 Oct 2(2) 49ndash55

bull Treatment and Outcomes for Gangliogliomas A Single-Center Review of 16 Patients

bull Jye Young Song Jeong Hoon Kim Young Hyun Cho Chang Jin Kim and Eun Jung Lee

bull Ganglioglioma is a rare and slowly growing benign tumor We investigated the outcomes of patients who underwent different combination treatments

bull Methods

bull Between 1998 and 2012 16 patients including 11 men and 5 women with a median age of 125 years (range 25-65 years) were treated for intracranial gangliogliomas at our institution The median follow-up period was 57 years (range 48 days-156 years) Fifteen cases were included in the outcome assessment because one patient was lost to follow-up Complete resection was achieved in 8 (53) patients Six (40) patients underwent incomplete resection with or without adjuvant radiotherapy and one patient with a brainstem tumor underwent only stereotactic biopsy

bull Results

bull Gangliogliomas predominantly affected young (875) male patients and most frequently presented with seizures (64) Of eight patients who underwent complete resection seven did not show recurrence whereas only three of six with incomplete resection showed no recurrence Four patients with recurrence received salvage treatments (two repeat surgeries and two radiosurgeries) A tumor control rate of 93 (1415) was achieved at the last follow-up No recurrence or malignant changes were observed after a median follow-up of 12 and 45 years in four patients who received gamma knife (GK) radiosurgery as adjuvant and salvage treatment

bull Conclusion

bull Complete resection produced the best outcomes and incomplete resection followed by adjuvant or salvage treatments showed favorable outcomes In patients who are not eligible for complete resection because of tumor location or potential neurologic deficits following surgery GK radiosurgery should be considered for the treatment of residual or recurrent tumors

10202016 41unusual giomas

Papillary tumours of pineal

bull WHO grade II AND IIIbull Relatively new edition to WHObull They are equal in both sexesbull Reach medical attention due to headache mental

status changes and altered visionbull Contain eosinophilic cells with distinctive

borders with large pleomorphic nucleibull Positive for cytokeratin synaptophysin stain

variably for GFAP vimentin S-100

10202016 unusual giomas 42

Imaging

bull Generally found to be diffusely enhancinglesion of pineal region

bull Post 3rd ventricle with HCPbull Appear well demarcated from thalamus and

cerebellum

10202016 unusual giomas 43

Mangement

bull Standard treatment is excisionbull Grow slowly and donot transform to

malignant tumorsbull Local recurrence is however possiblebull Radiotherapy appears to provide good control

in local recurrence

10202016 unusual giomas 44

Choroid Glioma Of Third Ventricle

bull WHO Grade IIbull Ist recognised by WHO in

1998bull Arise in the anterior third

ventriclebull Commonly come to

attention after HCPbull Appera to contain in 3rd

ventricle and thought toarise from ventricularsurface

bull Composed of GFAP andvimentin positive cells inmucinous matrix

bull Nuclei are uniform insize and shape

bull Indolent growthbull May show EGF receptor

positivitybull Some suggets these tumor

arise from the lminaterminalis andventricular tanycytes

10202016 45unusual giomas

Imaging

bull Commonly fill 3rd

ventriclebull Diffusely enhance with

contrastbull Appear to infiltrate the

thalamusbull Large tumors may be

difficult to differentiatefrom suprasellar masses

10202016 46unusual giomas

10202016 47unusual giomas

bull Surgical management is limited by locationbull Access through the dialted ventricle and lamina

terminalis may be challangingbull Subtotal resection is thus commonbull Role of adjuvant radiotherapy is unknown

10202016 48unusual giomas

bull Acta Neurochir (Wien) 2008 Nov150(11)1183-91 doi 101007s00701-008-0014-6 Epub

2008 Oct 21

bull Chordoid glioma of the third ventricle

bull Vanhauwaert DJ1 Clement F Van Dorpe J Deruytter MJKGROUND

bull Chordoid glioma is a rare tumour (World Health Organisation grade II) originating from the third ventricle

with both glial and chordoid features It was first described by Brat in 1998 Since there is no detailed

information available on the outcome after surgery and adjuvant treatment we reviewed the literature

bull METHODS

bull A literature search through PUBMED revealed 50 cases of chordoid glioma Most reports

were found in pathology journals Information on the postoperative course was sometimes

very limited We reviewed the available literature and studied in detail the presenting

symptoms mortality and postoperative complications in relation to the extent of resective

surgery as well as the importance of adjuvant treatment

bull CONCLUSIONS

bull Mortality in the immediate postoperative period is 32 and is higher after gross total

resection as compared to subtotal resection Non-fatal postoperative complications are

hypothalamic disorders and mental alterations Gross total resection is the treatment of

choice since no recurrence has been reported after macroscopically complete resection

but this is often difficult because of the location and adherence to the hypothalamus The

role of postoperative radiotherapy is uncertain There is some indication that radiosurgery

with or without conventional irradiation is superior to conventional radiation alone Planned

subtotal resection followed by stereotactic radiosurgery can be a safe and effective

alternative in a patient in whom gross total resection is considered to be too risky

There is no report on the use of chemotherapy in the treatment of chordoid gliomas More

information about the optimal treatment strategy is needed and more reports are also needed10202016 49unusual giomas

bull Neurologist 2011 Jan17(1)52bull Chordoid glioma a case report and literature reviewbull Liu WP1 Cheng JX Yi XC Zhen HN Fei Z Li Q Zhang Xbull chordoid glioma is a rare tumor (World Health Organization grade II) with both

glial and chordoid features often located in the suprasellar region and anterior third ventricle It was first described by Brat in 1998 Because there is no detailed information available from the clinical perspective we reviewed the literature

bull METHODS

bull a literature search through PUBMED and CNKI revealed 64 cases of chordoid glioma Information on the clinical course was very limited We reviewed the literature and studied the pathologic and imaging features postoperative mortality and morbidity in relation to surgical extension and approaches and the importance of adjuvant treatment

bull CONCLUSIONS bull mortality in the immediate postoperative period is 28 and postoperative

morbidity is 60 which are statistically higher after gross total resection as compared with subtotal resection Translamina terminalis approach is considered to be the best approach The current study cannot document that patients have longer survival and higher quality of life after gross total resection than subtotal resection The role of postoperative radiotherapy is uncertain and there is no report on the use of chemotherapy More information about the optimal treatment strategy is needed

10202016 50unusual giomas

Conclusion

bull They are identified by their unique morphologicalfeatures

bull Many of these have indolent course and identified byepilepsy

bull Potentail diagnosis should be considered in youngpatients with seizures and cortically based lesions

bull Correct diagnosis requires experiencedneuropathologisrt

bull As more experienced is gained more knowledgetowards better understanding shall provide betteroutcomes in these patients

10202016 unusual giomas 51

10202016 52unusual giomas

Content supportndash youmann neurosurgery textbook

Papillary tumours of pineal

bull WHO grade II AND IIIbull Relatively new edition to WHObull They are equal in both sexesbull Reach medical attention due to headache mental

status changes and altered visionbull Contain eosinophilic cells with distinctive

borders with large pleomorphic nucleibull Positive for cytokeratin synaptophysin stain

variably for GFAP vimentin S-100

10202016 unusual giomas 42

Imaging

bull Generally found to be diffusely enhancinglesion of pineal region

bull Post 3rd ventricle with HCPbull Appear well demarcated from thalamus and

cerebellum

10202016 unusual giomas 43

Mangement

bull Standard treatment is excisionbull Grow slowly and donot transform to

malignant tumorsbull Local recurrence is however possiblebull Radiotherapy appears to provide good control

in local recurrence

10202016 unusual giomas 44

Choroid Glioma Of Third Ventricle

bull WHO Grade IIbull Ist recognised by WHO in

1998bull Arise in the anterior third

ventriclebull Commonly come to

attention after HCPbull Appera to contain in 3rd

ventricle and thought toarise from ventricularsurface

bull Composed of GFAP andvimentin positive cells inmucinous matrix

bull Nuclei are uniform insize and shape

bull Indolent growthbull May show EGF receptor

positivitybull Some suggets these tumor

arise from the lminaterminalis andventricular tanycytes

10202016 45unusual giomas

Imaging

bull Commonly fill 3rd

ventriclebull Diffusely enhance with

contrastbull Appear to infiltrate the

thalamusbull Large tumors may be

difficult to differentiatefrom suprasellar masses

10202016 46unusual giomas

10202016 47unusual giomas

bull Surgical management is limited by locationbull Access through the dialted ventricle and lamina

terminalis may be challangingbull Subtotal resection is thus commonbull Role of adjuvant radiotherapy is unknown

10202016 48unusual giomas

bull Acta Neurochir (Wien) 2008 Nov150(11)1183-91 doi 101007s00701-008-0014-6 Epub

2008 Oct 21

bull Chordoid glioma of the third ventricle

bull Vanhauwaert DJ1 Clement F Van Dorpe J Deruytter MJKGROUND

bull Chordoid glioma is a rare tumour (World Health Organisation grade II) originating from the third ventricle

with both glial and chordoid features It was first described by Brat in 1998 Since there is no detailed

information available on the outcome after surgery and adjuvant treatment we reviewed the literature

bull METHODS

bull A literature search through PUBMED revealed 50 cases of chordoid glioma Most reports

were found in pathology journals Information on the postoperative course was sometimes

very limited We reviewed the available literature and studied in detail the presenting

symptoms mortality and postoperative complications in relation to the extent of resective

surgery as well as the importance of adjuvant treatment

bull CONCLUSIONS

bull Mortality in the immediate postoperative period is 32 and is higher after gross total

resection as compared to subtotal resection Non-fatal postoperative complications are

hypothalamic disorders and mental alterations Gross total resection is the treatment of

choice since no recurrence has been reported after macroscopically complete resection

but this is often difficult because of the location and adherence to the hypothalamus The

role of postoperative radiotherapy is uncertain There is some indication that radiosurgery

with or without conventional irradiation is superior to conventional radiation alone Planned

subtotal resection followed by stereotactic radiosurgery can be a safe and effective

alternative in a patient in whom gross total resection is considered to be too risky

There is no report on the use of chemotherapy in the treatment of chordoid gliomas More

information about the optimal treatment strategy is needed and more reports are also needed10202016 49unusual giomas

bull Neurologist 2011 Jan17(1)52bull Chordoid glioma a case report and literature reviewbull Liu WP1 Cheng JX Yi XC Zhen HN Fei Z Li Q Zhang Xbull chordoid glioma is a rare tumor (World Health Organization grade II) with both

glial and chordoid features often located in the suprasellar region and anterior third ventricle It was first described by Brat in 1998 Because there is no detailed information available from the clinical perspective we reviewed the literature

bull METHODS

bull a literature search through PUBMED and CNKI revealed 64 cases of chordoid glioma Information on the clinical course was very limited We reviewed the literature and studied the pathologic and imaging features postoperative mortality and morbidity in relation to surgical extension and approaches and the importance of adjuvant treatment

bull CONCLUSIONS bull mortality in the immediate postoperative period is 28 and postoperative

morbidity is 60 which are statistically higher after gross total resection as compared with subtotal resection Translamina terminalis approach is considered to be the best approach The current study cannot document that patients have longer survival and higher quality of life after gross total resection than subtotal resection The role of postoperative radiotherapy is uncertain and there is no report on the use of chemotherapy More information about the optimal treatment strategy is needed

10202016 50unusual giomas

Conclusion

bull They are identified by their unique morphologicalfeatures

bull Many of these have indolent course and identified byepilepsy

bull Potentail diagnosis should be considered in youngpatients with seizures and cortically based lesions

bull Correct diagnosis requires experiencedneuropathologisrt

bull As more experienced is gained more knowledgetowards better understanding shall provide betteroutcomes in these patients

10202016 unusual giomas 51

10202016 52unusual giomas

Content supportndash youmann neurosurgery textbook

Imaging

bull Generally found to be diffusely enhancinglesion of pineal region

bull Post 3rd ventricle with HCPbull Appear well demarcated from thalamus and

cerebellum

10202016 unusual giomas 43

Mangement

bull Standard treatment is excisionbull Grow slowly and donot transform to

malignant tumorsbull Local recurrence is however possiblebull Radiotherapy appears to provide good control

in local recurrence

10202016 unusual giomas 44

Choroid Glioma Of Third Ventricle

bull WHO Grade IIbull Ist recognised by WHO in

1998bull Arise in the anterior third

ventriclebull Commonly come to

attention after HCPbull Appera to contain in 3rd

ventricle and thought toarise from ventricularsurface

bull Composed of GFAP andvimentin positive cells inmucinous matrix

bull Nuclei are uniform insize and shape

bull Indolent growthbull May show EGF receptor

positivitybull Some suggets these tumor

arise from the lminaterminalis andventricular tanycytes

10202016 45unusual giomas

Imaging

bull Commonly fill 3rd

ventriclebull Diffusely enhance with

contrastbull Appear to infiltrate the

thalamusbull Large tumors may be

difficult to differentiatefrom suprasellar masses

10202016 46unusual giomas

10202016 47unusual giomas

bull Surgical management is limited by locationbull Access through the dialted ventricle and lamina

terminalis may be challangingbull Subtotal resection is thus commonbull Role of adjuvant radiotherapy is unknown

10202016 48unusual giomas

bull Acta Neurochir (Wien) 2008 Nov150(11)1183-91 doi 101007s00701-008-0014-6 Epub

2008 Oct 21

bull Chordoid glioma of the third ventricle

bull Vanhauwaert DJ1 Clement F Van Dorpe J Deruytter MJKGROUND

bull Chordoid glioma is a rare tumour (World Health Organisation grade II) originating from the third ventricle

with both glial and chordoid features It was first described by Brat in 1998 Since there is no detailed

information available on the outcome after surgery and adjuvant treatment we reviewed the literature

bull METHODS

bull A literature search through PUBMED revealed 50 cases of chordoid glioma Most reports

were found in pathology journals Information on the postoperative course was sometimes

very limited We reviewed the available literature and studied in detail the presenting

symptoms mortality and postoperative complications in relation to the extent of resective

surgery as well as the importance of adjuvant treatment

bull CONCLUSIONS

bull Mortality in the immediate postoperative period is 32 and is higher after gross total

resection as compared to subtotal resection Non-fatal postoperative complications are

hypothalamic disorders and mental alterations Gross total resection is the treatment of

choice since no recurrence has been reported after macroscopically complete resection

but this is often difficult because of the location and adherence to the hypothalamus The

role of postoperative radiotherapy is uncertain There is some indication that radiosurgery

with or without conventional irradiation is superior to conventional radiation alone Planned

subtotal resection followed by stereotactic radiosurgery can be a safe and effective

alternative in a patient in whom gross total resection is considered to be too risky

There is no report on the use of chemotherapy in the treatment of chordoid gliomas More

information about the optimal treatment strategy is needed and more reports are also needed10202016 49unusual giomas

bull Neurologist 2011 Jan17(1)52bull Chordoid glioma a case report and literature reviewbull Liu WP1 Cheng JX Yi XC Zhen HN Fei Z Li Q Zhang Xbull chordoid glioma is a rare tumor (World Health Organization grade II) with both

glial and chordoid features often located in the suprasellar region and anterior third ventricle It was first described by Brat in 1998 Because there is no detailed information available from the clinical perspective we reviewed the literature

bull METHODS

bull a literature search through PUBMED and CNKI revealed 64 cases of chordoid glioma Information on the clinical course was very limited We reviewed the literature and studied the pathologic and imaging features postoperative mortality and morbidity in relation to surgical extension and approaches and the importance of adjuvant treatment

bull CONCLUSIONS bull mortality in the immediate postoperative period is 28 and postoperative

morbidity is 60 which are statistically higher after gross total resection as compared with subtotal resection Translamina terminalis approach is considered to be the best approach The current study cannot document that patients have longer survival and higher quality of life after gross total resection than subtotal resection The role of postoperative radiotherapy is uncertain and there is no report on the use of chemotherapy More information about the optimal treatment strategy is needed

10202016 50unusual giomas

Conclusion

bull They are identified by their unique morphologicalfeatures

bull Many of these have indolent course and identified byepilepsy

bull Potentail diagnosis should be considered in youngpatients with seizures and cortically based lesions

bull Correct diagnosis requires experiencedneuropathologisrt

bull As more experienced is gained more knowledgetowards better understanding shall provide betteroutcomes in these patients

10202016 unusual giomas 51

10202016 52unusual giomas

Content supportndash youmann neurosurgery textbook

Mangement

bull Standard treatment is excisionbull Grow slowly and donot transform to

malignant tumorsbull Local recurrence is however possiblebull Radiotherapy appears to provide good control

in local recurrence

10202016 unusual giomas 44

Choroid Glioma Of Third Ventricle

bull WHO Grade IIbull Ist recognised by WHO in

1998bull Arise in the anterior third

ventriclebull Commonly come to

attention after HCPbull Appera to contain in 3rd

ventricle and thought toarise from ventricularsurface

bull Composed of GFAP andvimentin positive cells inmucinous matrix

bull Nuclei are uniform insize and shape

bull Indolent growthbull May show EGF receptor

positivitybull Some suggets these tumor

arise from the lminaterminalis andventricular tanycytes

10202016 45unusual giomas

Imaging

bull Commonly fill 3rd

ventriclebull Diffusely enhance with

contrastbull Appear to infiltrate the

thalamusbull Large tumors may be

difficult to differentiatefrom suprasellar masses

10202016 46unusual giomas

10202016 47unusual giomas

bull Surgical management is limited by locationbull Access through the dialted ventricle and lamina

terminalis may be challangingbull Subtotal resection is thus commonbull Role of adjuvant radiotherapy is unknown

10202016 48unusual giomas

bull Acta Neurochir (Wien) 2008 Nov150(11)1183-91 doi 101007s00701-008-0014-6 Epub

2008 Oct 21

bull Chordoid glioma of the third ventricle

bull Vanhauwaert DJ1 Clement F Van Dorpe J Deruytter MJKGROUND

bull Chordoid glioma is a rare tumour (World Health Organisation grade II) originating from the third ventricle

with both glial and chordoid features It was first described by Brat in 1998 Since there is no detailed

information available on the outcome after surgery and adjuvant treatment we reviewed the literature

bull METHODS

bull A literature search through PUBMED revealed 50 cases of chordoid glioma Most reports

were found in pathology journals Information on the postoperative course was sometimes

very limited We reviewed the available literature and studied in detail the presenting

symptoms mortality and postoperative complications in relation to the extent of resective

surgery as well as the importance of adjuvant treatment

bull CONCLUSIONS

bull Mortality in the immediate postoperative period is 32 and is higher after gross total

resection as compared to subtotal resection Non-fatal postoperative complications are

hypothalamic disorders and mental alterations Gross total resection is the treatment of

choice since no recurrence has been reported after macroscopically complete resection

but this is often difficult because of the location and adherence to the hypothalamus The

role of postoperative radiotherapy is uncertain There is some indication that radiosurgery

with or without conventional irradiation is superior to conventional radiation alone Planned

subtotal resection followed by stereotactic radiosurgery can be a safe and effective

alternative in a patient in whom gross total resection is considered to be too risky

There is no report on the use of chemotherapy in the treatment of chordoid gliomas More

information about the optimal treatment strategy is needed and more reports are also needed10202016 49unusual giomas

bull Neurologist 2011 Jan17(1)52bull Chordoid glioma a case report and literature reviewbull Liu WP1 Cheng JX Yi XC Zhen HN Fei Z Li Q Zhang Xbull chordoid glioma is a rare tumor (World Health Organization grade II) with both

glial and chordoid features often located in the suprasellar region and anterior third ventricle It was first described by Brat in 1998 Because there is no detailed information available from the clinical perspective we reviewed the literature

bull METHODS

bull a literature search through PUBMED and CNKI revealed 64 cases of chordoid glioma Information on the clinical course was very limited We reviewed the literature and studied the pathologic and imaging features postoperative mortality and morbidity in relation to surgical extension and approaches and the importance of adjuvant treatment

bull CONCLUSIONS bull mortality in the immediate postoperative period is 28 and postoperative

morbidity is 60 which are statistically higher after gross total resection as compared with subtotal resection Translamina terminalis approach is considered to be the best approach The current study cannot document that patients have longer survival and higher quality of life after gross total resection than subtotal resection The role of postoperative radiotherapy is uncertain and there is no report on the use of chemotherapy More information about the optimal treatment strategy is needed

10202016 50unusual giomas

Conclusion

bull They are identified by their unique morphologicalfeatures

bull Many of these have indolent course and identified byepilepsy

bull Potentail diagnosis should be considered in youngpatients with seizures and cortically based lesions

bull Correct diagnosis requires experiencedneuropathologisrt

bull As more experienced is gained more knowledgetowards better understanding shall provide betteroutcomes in these patients

10202016 unusual giomas 51

10202016 52unusual giomas

Content supportndash youmann neurosurgery textbook

Choroid Glioma Of Third Ventricle

bull WHO Grade IIbull Ist recognised by WHO in

1998bull Arise in the anterior third

ventriclebull Commonly come to

attention after HCPbull Appera to contain in 3rd

ventricle and thought toarise from ventricularsurface

bull Composed of GFAP andvimentin positive cells inmucinous matrix

bull Nuclei are uniform insize and shape

bull Indolent growthbull May show EGF receptor

positivitybull Some suggets these tumor

arise from the lminaterminalis andventricular tanycytes

10202016 45unusual giomas

Imaging

bull Commonly fill 3rd

ventriclebull Diffusely enhance with

contrastbull Appear to infiltrate the

thalamusbull Large tumors may be

difficult to differentiatefrom suprasellar masses

10202016 46unusual giomas

10202016 47unusual giomas

bull Surgical management is limited by locationbull Access through the dialted ventricle and lamina

terminalis may be challangingbull Subtotal resection is thus commonbull Role of adjuvant radiotherapy is unknown

10202016 48unusual giomas

bull Acta Neurochir (Wien) 2008 Nov150(11)1183-91 doi 101007s00701-008-0014-6 Epub

2008 Oct 21

bull Chordoid glioma of the third ventricle

bull Vanhauwaert DJ1 Clement F Van Dorpe J Deruytter MJKGROUND

bull Chordoid glioma is a rare tumour (World Health Organisation grade II) originating from the third ventricle

with both glial and chordoid features It was first described by Brat in 1998 Since there is no detailed

information available on the outcome after surgery and adjuvant treatment we reviewed the literature

bull METHODS

bull A literature search through PUBMED revealed 50 cases of chordoid glioma Most reports

were found in pathology journals Information on the postoperative course was sometimes

very limited We reviewed the available literature and studied in detail the presenting

symptoms mortality and postoperative complications in relation to the extent of resective

surgery as well as the importance of adjuvant treatment

bull CONCLUSIONS

bull Mortality in the immediate postoperative period is 32 and is higher after gross total

resection as compared to subtotal resection Non-fatal postoperative complications are

hypothalamic disorders and mental alterations Gross total resection is the treatment of

choice since no recurrence has been reported after macroscopically complete resection

but this is often difficult because of the location and adherence to the hypothalamus The

role of postoperative radiotherapy is uncertain There is some indication that radiosurgery

with or without conventional irradiation is superior to conventional radiation alone Planned

subtotal resection followed by stereotactic radiosurgery can be a safe and effective

alternative in a patient in whom gross total resection is considered to be too risky

There is no report on the use of chemotherapy in the treatment of chordoid gliomas More

information about the optimal treatment strategy is needed and more reports are also needed10202016 49unusual giomas

bull Neurologist 2011 Jan17(1)52bull Chordoid glioma a case report and literature reviewbull Liu WP1 Cheng JX Yi XC Zhen HN Fei Z Li Q Zhang Xbull chordoid glioma is a rare tumor (World Health Organization grade II) with both

glial and chordoid features often located in the suprasellar region and anterior third ventricle It was first described by Brat in 1998 Because there is no detailed information available from the clinical perspective we reviewed the literature

bull METHODS

bull a literature search through PUBMED and CNKI revealed 64 cases of chordoid glioma Information on the clinical course was very limited We reviewed the literature and studied the pathologic and imaging features postoperative mortality and morbidity in relation to surgical extension and approaches and the importance of adjuvant treatment

bull CONCLUSIONS bull mortality in the immediate postoperative period is 28 and postoperative

morbidity is 60 which are statistically higher after gross total resection as compared with subtotal resection Translamina terminalis approach is considered to be the best approach The current study cannot document that patients have longer survival and higher quality of life after gross total resection than subtotal resection The role of postoperative radiotherapy is uncertain and there is no report on the use of chemotherapy More information about the optimal treatment strategy is needed

10202016 50unusual giomas

Conclusion

bull They are identified by their unique morphologicalfeatures

bull Many of these have indolent course and identified byepilepsy

bull Potentail diagnosis should be considered in youngpatients with seizures and cortically based lesions

bull Correct diagnosis requires experiencedneuropathologisrt

bull As more experienced is gained more knowledgetowards better understanding shall provide betteroutcomes in these patients

10202016 unusual giomas 51

10202016 52unusual giomas

Content supportndash youmann neurosurgery textbook

Imaging

bull Commonly fill 3rd

ventriclebull Diffusely enhance with

contrastbull Appear to infiltrate the

thalamusbull Large tumors may be

difficult to differentiatefrom suprasellar masses

10202016 46unusual giomas

10202016 47unusual giomas

bull Surgical management is limited by locationbull Access through the dialted ventricle and lamina

terminalis may be challangingbull Subtotal resection is thus commonbull Role of adjuvant radiotherapy is unknown

10202016 48unusual giomas

bull Acta Neurochir (Wien) 2008 Nov150(11)1183-91 doi 101007s00701-008-0014-6 Epub

2008 Oct 21

bull Chordoid glioma of the third ventricle

bull Vanhauwaert DJ1 Clement F Van Dorpe J Deruytter MJKGROUND

bull Chordoid glioma is a rare tumour (World Health Organisation grade II) originating from the third ventricle

with both glial and chordoid features It was first described by Brat in 1998 Since there is no detailed

information available on the outcome after surgery and adjuvant treatment we reviewed the literature

bull METHODS

bull A literature search through PUBMED revealed 50 cases of chordoid glioma Most reports

were found in pathology journals Information on the postoperative course was sometimes

very limited We reviewed the available literature and studied in detail the presenting

symptoms mortality and postoperative complications in relation to the extent of resective

surgery as well as the importance of adjuvant treatment

bull CONCLUSIONS

bull Mortality in the immediate postoperative period is 32 and is higher after gross total

resection as compared to subtotal resection Non-fatal postoperative complications are

hypothalamic disorders and mental alterations Gross total resection is the treatment of

choice since no recurrence has been reported after macroscopically complete resection

but this is often difficult because of the location and adherence to the hypothalamus The

role of postoperative radiotherapy is uncertain There is some indication that radiosurgery

with or without conventional irradiation is superior to conventional radiation alone Planned

subtotal resection followed by stereotactic radiosurgery can be a safe and effective

alternative in a patient in whom gross total resection is considered to be too risky

There is no report on the use of chemotherapy in the treatment of chordoid gliomas More

information about the optimal treatment strategy is needed and more reports are also needed10202016 49unusual giomas

bull Neurologist 2011 Jan17(1)52bull Chordoid glioma a case report and literature reviewbull Liu WP1 Cheng JX Yi XC Zhen HN Fei Z Li Q Zhang Xbull chordoid glioma is a rare tumor (World Health Organization grade II) with both

glial and chordoid features often located in the suprasellar region and anterior third ventricle It was first described by Brat in 1998 Because there is no detailed information available from the clinical perspective we reviewed the literature

bull METHODS

bull a literature search through PUBMED and CNKI revealed 64 cases of chordoid glioma Information on the clinical course was very limited We reviewed the literature and studied the pathologic and imaging features postoperative mortality and morbidity in relation to surgical extension and approaches and the importance of adjuvant treatment

bull CONCLUSIONS bull mortality in the immediate postoperative period is 28 and postoperative

morbidity is 60 which are statistically higher after gross total resection as compared with subtotal resection Translamina terminalis approach is considered to be the best approach The current study cannot document that patients have longer survival and higher quality of life after gross total resection than subtotal resection The role of postoperative radiotherapy is uncertain and there is no report on the use of chemotherapy More information about the optimal treatment strategy is needed

10202016 50unusual giomas

Conclusion

bull They are identified by their unique morphologicalfeatures

bull Many of these have indolent course and identified byepilepsy

bull Potentail diagnosis should be considered in youngpatients with seizures and cortically based lesions

bull Correct diagnosis requires experiencedneuropathologisrt

bull As more experienced is gained more knowledgetowards better understanding shall provide betteroutcomes in these patients

10202016 unusual giomas 51

10202016 52unusual giomas

Content supportndash youmann neurosurgery textbook

10202016 47unusual giomas

bull Surgical management is limited by locationbull Access through the dialted ventricle and lamina

terminalis may be challangingbull Subtotal resection is thus commonbull Role of adjuvant radiotherapy is unknown

10202016 48unusual giomas

bull Acta Neurochir (Wien) 2008 Nov150(11)1183-91 doi 101007s00701-008-0014-6 Epub

2008 Oct 21

bull Chordoid glioma of the third ventricle

bull Vanhauwaert DJ1 Clement F Van Dorpe J Deruytter MJKGROUND

bull Chordoid glioma is a rare tumour (World Health Organisation grade II) originating from the third ventricle

with both glial and chordoid features It was first described by Brat in 1998 Since there is no detailed

information available on the outcome after surgery and adjuvant treatment we reviewed the literature

bull METHODS

bull A literature search through PUBMED revealed 50 cases of chordoid glioma Most reports

were found in pathology journals Information on the postoperative course was sometimes

very limited We reviewed the available literature and studied in detail the presenting

symptoms mortality and postoperative complications in relation to the extent of resective

surgery as well as the importance of adjuvant treatment

bull CONCLUSIONS

bull Mortality in the immediate postoperative period is 32 and is higher after gross total

resection as compared to subtotal resection Non-fatal postoperative complications are

hypothalamic disorders and mental alterations Gross total resection is the treatment of

choice since no recurrence has been reported after macroscopically complete resection

but this is often difficult because of the location and adherence to the hypothalamus The

role of postoperative radiotherapy is uncertain There is some indication that radiosurgery

with or without conventional irradiation is superior to conventional radiation alone Planned

subtotal resection followed by stereotactic radiosurgery can be a safe and effective

alternative in a patient in whom gross total resection is considered to be too risky

There is no report on the use of chemotherapy in the treatment of chordoid gliomas More

information about the optimal treatment strategy is needed and more reports are also needed10202016 49unusual giomas

bull Neurologist 2011 Jan17(1)52bull Chordoid glioma a case report and literature reviewbull Liu WP1 Cheng JX Yi XC Zhen HN Fei Z Li Q Zhang Xbull chordoid glioma is a rare tumor (World Health Organization grade II) with both

glial and chordoid features often located in the suprasellar region and anterior third ventricle It was first described by Brat in 1998 Because there is no detailed information available from the clinical perspective we reviewed the literature

bull METHODS

bull a literature search through PUBMED and CNKI revealed 64 cases of chordoid glioma Information on the clinical course was very limited We reviewed the literature and studied the pathologic and imaging features postoperative mortality and morbidity in relation to surgical extension and approaches and the importance of adjuvant treatment

bull CONCLUSIONS bull mortality in the immediate postoperative period is 28 and postoperative

morbidity is 60 which are statistically higher after gross total resection as compared with subtotal resection Translamina terminalis approach is considered to be the best approach The current study cannot document that patients have longer survival and higher quality of life after gross total resection than subtotal resection The role of postoperative radiotherapy is uncertain and there is no report on the use of chemotherapy More information about the optimal treatment strategy is needed

10202016 50unusual giomas

Conclusion

bull They are identified by their unique morphologicalfeatures

bull Many of these have indolent course and identified byepilepsy

bull Potentail diagnosis should be considered in youngpatients with seizures and cortically based lesions

bull Correct diagnosis requires experiencedneuropathologisrt

bull As more experienced is gained more knowledgetowards better understanding shall provide betteroutcomes in these patients

10202016 unusual giomas 51

10202016 52unusual giomas

Content supportndash youmann neurosurgery textbook

bull Surgical management is limited by locationbull Access through the dialted ventricle and lamina

terminalis may be challangingbull Subtotal resection is thus commonbull Role of adjuvant radiotherapy is unknown

10202016 48unusual giomas

bull Acta Neurochir (Wien) 2008 Nov150(11)1183-91 doi 101007s00701-008-0014-6 Epub

2008 Oct 21

bull Chordoid glioma of the third ventricle

bull Vanhauwaert DJ1 Clement F Van Dorpe J Deruytter MJKGROUND

bull Chordoid glioma is a rare tumour (World Health Organisation grade II) originating from the third ventricle

with both glial and chordoid features It was first described by Brat in 1998 Since there is no detailed

information available on the outcome after surgery and adjuvant treatment we reviewed the literature

bull METHODS

bull A literature search through PUBMED revealed 50 cases of chordoid glioma Most reports

were found in pathology journals Information on the postoperative course was sometimes

very limited We reviewed the available literature and studied in detail the presenting

symptoms mortality and postoperative complications in relation to the extent of resective

surgery as well as the importance of adjuvant treatment

bull CONCLUSIONS

bull Mortality in the immediate postoperative period is 32 and is higher after gross total

resection as compared to subtotal resection Non-fatal postoperative complications are

hypothalamic disorders and mental alterations Gross total resection is the treatment of

choice since no recurrence has been reported after macroscopically complete resection

but this is often difficult because of the location and adherence to the hypothalamus The

role of postoperative radiotherapy is uncertain There is some indication that radiosurgery

with or without conventional irradiation is superior to conventional radiation alone Planned

subtotal resection followed by stereotactic radiosurgery can be a safe and effective

alternative in a patient in whom gross total resection is considered to be too risky

There is no report on the use of chemotherapy in the treatment of chordoid gliomas More

information about the optimal treatment strategy is needed and more reports are also needed10202016 49unusual giomas

bull Neurologist 2011 Jan17(1)52bull Chordoid glioma a case report and literature reviewbull Liu WP1 Cheng JX Yi XC Zhen HN Fei Z Li Q Zhang Xbull chordoid glioma is a rare tumor (World Health Organization grade II) with both

glial and chordoid features often located in the suprasellar region and anterior third ventricle It was first described by Brat in 1998 Because there is no detailed information available from the clinical perspective we reviewed the literature

bull METHODS

bull a literature search through PUBMED and CNKI revealed 64 cases of chordoid glioma Information on the clinical course was very limited We reviewed the literature and studied the pathologic and imaging features postoperative mortality and morbidity in relation to surgical extension and approaches and the importance of adjuvant treatment

bull CONCLUSIONS bull mortality in the immediate postoperative period is 28 and postoperative

morbidity is 60 which are statistically higher after gross total resection as compared with subtotal resection Translamina terminalis approach is considered to be the best approach The current study cannot document that patients have longer survival and higher quality of life after gross total resection than subtotal resection The role of postoperative radiotherapy is uncertain and there is no report on the use of chemotherapy More information about the optimal treatment strategy is needed

10202016 50unusual giomas

Conclusion

bull They are identified by their unique morphologicalfeatures

bull Many of these have indolent course and identified byepilepsy

bull Potentail diagnosis should be considered in youngpatients with seizures and cortically based lesions

bull Correct diagnosis requires experiencedneuropathologisrt

bull As more experienced is gained more knowledgetowards better understanding shall provide betteroutcomes in these patients

10202016 unusual giomas 51

10202016 52unusual giomas

Content supportndash youmann neurosurgery textbook

bull Acta Neurochir (Wien) 2008 Nov150(11)1183-91 doi 101007s00701-008-0014-6 Epub

2008 Oct 21

bull Chordoid glioma of the third ventricle

bull Vanhauwaert DJ1 Clement F Van Dorpe J Deruytter MJKGROUND

bull Chordoid glioma is a rare tumour (World Health Organisation grade II) originating from the third ventricle

with both glial and chordoid features It was first described by Brat in 1998 Since there is no detailed

information available on the outcome after surgery and adjuvant treatment we reviewed the literature

bull METHODS

bull A literature search through PUBMED revealed 50 cases of chordoid glioma Most reports

were found in pathology journals Information on the postoperative course was sometimes

very limited We reviewed the available literature and studied in detail the presenting

symptoms mortality and postoperative complications in relation to the extent of resective

surgery as well as the importance of adjuvant treatment

bull CONCLUSIONS

bull Mortality in the immediate postoperative period is 32 and is higher after gross total

resection as compared to subtotal resection Non-fatal postoperative complications are

hypothalamic disorders and mental alterations Gross total resection is the treatment of

choice since no recurrence has been reported after macroscopically complete resection

but this is often difficult because of the location and adherence to the hypothalamus The

role of postoperative radiotherapy is uncertain There is some indication that radiosurgery

with or without conventional irradiation is superior to conventional radiation alone Planned

subtotal resection followed by stereotactic radiosurgery can be a safe and effective

alternative in a patient in whom gross total resection is considered to be too risky

There is no report on the use of chemotherapy in the treatment of chordoid gliomas More

information about the optimal treatment strategy is needed and more reports are also needed10202016 49unusual giomas

bull Neurologist 2011 Jan17(1)52bull Chordoid glioma a case report and literature reviewbull Liu WP1 Cheng JX Yi XC Zhen HN Fei Z Li Q Zhang Xbull chordoid glioma is a rare tumor (World Health Organization grade II) with both

glial and chordoid features often located in the suprasellar region and anterior third ventricle It was first described by Brat in 1998 Because there is no detailed information available from the clinical perspective we reviewed the literature

bull METHODS

bull a literature search through PUBMED and CNKI revealed 64 cases of chordoid glioma Information on the clinical course was very limited We reviewed the literature and studied the pathologic and imaging features postoperative mortality and morbidity in relation to surgical extension and approaches and the importance of adjuvant treatment

bull CONCLUSIONS bull mortality in the immediate postoperative period is 28 and postoperative

morbidity is 60 which are statistically higher after gross total resection as compared with subtotal resection Translamina terminalis approach is considered to be the best approach The current study cannot document that patients have longer survival and higher quality of life after gross total resection than subtotal resection The role of postoperative radiotherapy is uncertain and there is no report on the use of chemotherapy More information about the optimal treatment strategy is needed

10202016 50unusual giomas

Conclusion

bull They are identified by their unique morphologicalfeatures

bull Many of these have indolent course and identified byepilepsy

bull Potentail diagnosis should be considered in youngpatients with seizures and cortically based lesions

bull Correct diagnosis requires experiencedneuropathologisrt

bull As more experienced is gained more knowledgetowards better understanding shall provide betteroutcomes in these patients

10202016 unusual giomas 51

10202016 52unusual giomas

Content supportndash youmann neurosurgery textbook

bull Neurologist 2011 Jan17(1)52bull Chordoid glioma a case report and literature reviewbull Liu WP1 Cheng JX Yi XC Zhen HN Fei Z Li Q Zhang Xbull chordoid glioma is a rare tumor (World Health Organization grade II) with both

glial and chordoid features often located in the suprasellar region and anterior third ventricle It was first described by Brat in 1998 Because there is no detailed information available from the clinical perspective we reviewed the literature

bull METHODS

bull a literature search through PUBMED and CNKI revealed 64 cases of chordoid glioma Information on the clinical course was very limited We reviewed the literature and studied the pathologic and imaging features postoperative mortality and morbidity in relation to surgical extension and approaches and the importance of adjuvant treatment

bull CONCLUSIONS bull mortality in the immediate postoperative period is 28 and postoperative

morbidity is 60 which are statistically higher after gross total resection as compared with subtotal resection Translamina terminalis approach is considered to be the best approach The current study cannot document that patients have longer survival and higher quality of life after gross total resection than subtotal resection The role of postoperative radiotherapy is uncertain and there is no report on the use of chemotherapy More information about the optimal treatment strategy is needed

10202016 50unusual giomas

Conclusion

bull They are identified by their unique morphologicalfeatures

bull Many of these have indolent course and identified byepilepsy

bull Potentail diagnosis should be considered in youngpatients with seizures and cortically based lesions

bull Correct diagnosis requires experiencedneuropathologisrt

bull As more experienced is gained more knowledgetowards better understanding shall provide betteroutcomes in these patients

10202016 unusual giomas 51

10202016 52unusual giomas

Content supportndash youmann neurosurgery textbook

Conclusion

bull They are identified by their unique morphologicalfeatures

bull Many of these have indolent course and identified byepilepsy

bull Potentail diagnosis should be considered in youngpatients with seizures and cortically based lesions

bull Correct diagnosis requires experiencedneuropathologisrt

bull As more experienced is gained more knowledgetowards better understanding shall provide betteroutcomes in these patients

10202016 unusual giomas 51

10202016 52unusual giomas

Content supportndash youmann neurosurgery textbook

10202016 52unusual giomas

Content supportndash youmann neurosurgery textbook