Man Bahadur Paudyal

Introduction IClassification:

A. Based on origin of tumor1. Primary 2. Secondary (Metastatic) B. Based on nature of tumor1. Benign: well-encapsulated, slow-growing non-infiltrative, low tendency to invade 2. Malignant: non-encapsulated, rapid-growing highly infiltrative.

Introduction IIOccurrence

CNS Tumor: 10% of all tumors Brain (80%) Spinal Cord (20%)

Ultimate outcome: increased ICP Mass effect tumor perifocal edema intratumoral hge Direct compressioninfiltration CSF pathway obstructionFocal effects CN deficits

Clinical Manifestation

Depends on the location of the tumor.With supratentorial tumors:

1. Seizure2. Mental changes (memory loss, behavior changes)3. Focal deficits(visual field deficit, paralysis)

With infratentorial tumors:1. CN palsies2. Cerebellar signs (ataxia, dysmetria, tremor, nystagmus, diadodiskinesia)3. Obstructive hydrocephalus

Clinical Syndromes Frontal lobe syndrome

1) Mental/ personality changes2) Incontinence3) Speech disorders4) Paralysis

Temporal lobe syndrome1) Aphasia2) Psychomotor seizures3) Visual field changes (hemianopia)

Parietal lobe syndrome I

I. With dominant hemisphere1) Gerstman’s syndrome

a) agraphia without alexia (can read but not write)

b) left to right confusionc) digital agnosia ( can not name

fingers)d) acalculia (can not do simple

calculation)2) Language disorder3) Tactile agnosia4) Ideomotor apraxia

Parietal Syndromes II

II. With non-dominant hemisphere1) Topographic memory loss2) Anosognosia3) Dressing apraxia

III. With either1) Focal seizures2) Agnosia3) Sensory changes4) Dyslexia

General Clinical Manifestation1. Severe progressive headache, worst in morning

2. Vomiting, projectile, without prenauseate phase3. Seizures (generalized/focal)4. Altered sensorium5. Changes in mentation6. Abnormal sensations7. Increased ICP8. Papilledema9. Central herniation with brainstem dysfunction10. Compression of brain parenchyma/blood vessels/CSF

path(1) Ischemia (2) Infarction (3) Hydrocephalus(4) Edema (5) Hemorrhage

11. Suture separation in children < 5 yr12. Bony erosion

Classification of CNS TumorIn adults, the majority are

supratentorial and of metastatic type; whereas in children, infratentorial tumors are more common.

Common Pediatric Intracranial Tumors

I. MedulloblastomaII. EpendymomaIII. Cerebral AstrocytomaIV. Cerebellar AstrocytomaV. Brainstem Glioma (BSG)VI. Craniopharyngioma (CP)

MedulloblastomaMost common malignant pediatric brain tumor (15 ~ 20%)Arises from cerebellar vermis & apex of IV ventricle roofPredispose to early obstruction of CSF pathwaysCSF seedlings

CT-scan: sold midline enhancing lesion

MRI:Banana signHighly radiosensitive/ moderately chemosensitive

TreatmentSurgery Suboccipital craniectomy+Excision ShuntVentriculostomy Definitive surgeryPostoperative radiation/ chemotherapy

PrognosisPoor prognosis Younger age < 4 yrDisseminationUnable to perform total resectionHistological differentiationWithout treatment, survival 1 ~ 2 yrsWith treatment, 5 & 10 yr survival 56% & 43%

EpendymomaUsually benign but may be inoperable due to

locationIntracranial location is usually infratentorial and in

spine, especially in filum teminalis (myxopapillary)

May seed along CSF pathways/ occur along neuraxis

CT/MRI: commonly calcified, inhomogeneous on T1WI and exophytic component is high signal in T2WI

TreatmentSurgery & postoperative radiation

PrognosisWorse due to propensity to invade obex5 yr survival 41%

Cerebral AstrocytomaTypes:

Low gr ( I + II ): less malignant better prognosisHigh gr ( III+IV ): highly malignant poor outcome

Usually involve:Frontal Basal ganglia ThalamusMidline structures

Butterfly glioma: arising from corpus callosum with bifrontal lobe involvement

Arises from neuro-ectodermal tissueTreatment: Surgery/Biopsy

Postoperative radio/chemotherapyPrognosis: Average survival < 1 yr in high grade

IV. Cerebellar astrocytoma Usually benign & cystic Usually present during 2nd decade Usually involves pons, midbrain & medulla Does not seed along CSF pathway

Pilocytic typeoccur in younger age & have better prognosisRadiographically, cystic with mural nodulePathologically, compact/loose astrocytes with Rosenthal fibres

Treatment: Surgery + Postoperative radiotherapy

Prognosis: Long-term survival possible with combined

V. Brainstem Glioma (BSG)

Slow-growing & highly malignant with poor prognosis

Usually presents with multiple CN palsies & long tract signs

Treatment: Surgery + postoperative radiotherapy

Prognosis:With RXT, survival 4 yr Without RXT, only 1 yr

Craniopharyngioma (CP) Benign suprasellar lesion Arises from rakhte’s remnants May be prechiasmatic/retrochiasmatic Causes pituitary dysfunction, visual field deficits CT/MRI: almost all have solid & cystic

componentsSpoke & Wheel appearance

Treatment: Surgery: Subtemporal/subfrontal/transsphenoidal Postoperative XRT

Prognosis: Favorable if totally resected

Common intracranial tumors in adults I. GliomaII. MeningiomaIII. Vestibular Schwanoma (Acoustic

neuroma)IV. Pituitary AdenomaV. Miscellaneous tumors

I. Glioma

All tumors that arise from neuroglial cells Consists of (1) Astrocytoma (2)

Oligodendroglioma

Oligodendroglioma (Oligo)1. Slow-growing & often calcified2. Frequently presents with seizures3. Occurs in cerebral hemispheres4. Fried egg appearance in LP microscopic view

Meningioma

Usually benign, slow-growing, frequently calcified & extra-axial

Commonly seen in middle-aged womenArising from arachnoid layerUsually located supratentorial along falx,

convexity/sphenoidTendency to compress than infiltrate brain

parenchymaClassic pathological findings is psammomna

bodiesUsually cured if completely removed which is

usually impossibleMay be highly vascular (angioblastic)

CT-scan: Homogeneously enhancing lesion with broad base attachment along durausually with little/ no perifocal edema

MRI: Dural tailTreatment: Surgery & Postoperative XRTPrognosis:

Favorable with total resection, maybe recurrentIn totally resected case, recurrent rate 15%With partial resection upto 85% after 5 yr

Pituitary AdenomaArises from anterior part of pituitary (Adenopypophysis)Type: Microadenoma < 1 cm Macroadenoma

Functional Non-functionalCauses

Compression of optic chiasma bitemporal hemianopiaCompression of cavernous sinus CN palsy(III, IV VI, V1,2)Endocrinologic disturbancesACTH Cushing’s diseasePRL Amenorrhea-galactorrhea syndromeGH Gigantism (Children) Acromegaly (adult)

Apoplexy: Abrupt onset of neurologic deterioration due to expanding mass as result of hemorrhageCauses visual deterioration, ophthalmoplegia, reduced MS & pituitary hypofunction

Acoustic SchwanomaBenign, usually unilateral or maybe part of MEN (bilateral)= NF2

Arises from vestibular branch of 8th CNCauses compression than infiltrationClassic signs: insidious/progressive

Early: Decreased hearing High-pitch tinnitusDizziness DysequilibriumLate:Hemifacial numbness (CN V palsy)Facial asymmetry, lidlag ( CN VII palsy)

Pathology: benign with Antoni A & BCT-scan: erosion/enlargement of IAC (bone

window)Ice cream cone

MRI: Round/oval enhancing lesion centered on IAC

Treatment: Surgery Retrosigmoid, Translabyrinthine, Subtemporal

Miscellaneous Tumors IColloid cyst

Classically occur in III ventricle blocking Foramen of MonroCauses obstructive hydrocephalusSlow-growing, benign lesionTreatment: Surgery( Trancallous/Transcortical Vs Endoscopic)VP Shunt

HemangioblastomaMost common primary intra-axial tumor in adult posterior fossaMay be part of von Hippel Lindau diseaseMay be associated with erythrocytosis

Cerebral LymphomaCT/MRI: homogeneously enhancing lesion in central gray matterFluffy cotton ball appearanceMay present with multiple CN palsiesExtremely responsive to steroids ( ghost tumors)Diagnosis highly likely if uveitis is present

Miscellaneous Tumors IIChordoma

Benign, highly recurrent, slow-growing, locally aggressiveGenerally radio/chemoresistentArises from remnants of primitive notochordCranially, found in sphenooccipital region (clivus) & in sacrococcygeal region in spine

Chordosarcoma: Arises from paramedial region

Cerebral MetastasisMost common brain tumor seen clinicallySources: Adults: Lung, breast, kidney(, GI, melanoma, thyroidChildren: Neuroblastoma, rhabdomyosarcoma, Wilm’s High grade glioma, medullo, ependymoma, pineal tumorLocation: parenchyma/leptomeninges

80% in cerebral hemispheres, mostly parietal lobe

Pseudotumor Cerebri (Benign/Idiopathic Intracranial Hypertension)Increased ICP papilledema without intracranial

mass, hydrocephalus or normal CT/MRI

Usually self-limited, easily recurring, chronicPreventable cause of blindness from optic atrophy

TreatmentFluid/salt restrictionDiamox 250 mg PO q8Lasix up to 160 mg/dSteroid Dexamethasone 4 mg PO q6

Prednisolone 40 ~ 60 mg PO qd

Surgery Serial LP, LP shuntOptic N sheath decompression

Treatment in General

I. Surgery if accessibleII. Radiation if

radiosensitiveIII. Chemotherapy if chemosensitive

Prognosis depends on type & location

Introduction

15% of primary CNS tumors Cranial:Spinal astrocytoma = 10:1 Cranial: Spinal ependymoma = 3 ~20:1

Most primary spinal tumors are benign

Compression symptoms

Types of Spinal Tumors

Extradural: (55%) Intradural Extramedullary: (40%)

Meningioma & neurofibroma Intramedullary: (5%)

Extradural Spinal Tumors

Metastatic Lymphoma, lung, breast, prostate

Primary Chordoma, neurofibroma,

osteoblastoma, hemangioma Meningioma

Intradural Extramedullary Spinal Tumor Meningioma neurofibroma

Intramedullary Spinal Tumor Astrocytoma 30% Ependymoma 30% Other 30%

Glioblastoma, dermoid, epidermoid, lipoma

Clinical Presentation

Pain Weakness Paresthesia Sphincter disturbance Other

Deformity: scoliosis/ torticollis SAH Mass

Diagnosis

Plain X-ray LP Myelogram CT MRI Angiography

Differential Diagnosis

Vascular malformation Demyelinating diseases ( MS) Transverse myelitis Paraneoplastic myelopathy