Intradural Spinal Neoplasms · Spinal Cord Anatomy 31 pairs of spinal nerves 8 cervical 12 thoracic...

IntraduralIntradural Spinal Spinal NeoplasmsNeoplasmsJohn K. John K. BirknesBirknes, M.D., M.D.

Department of NeurosurgeryDepartment of NeurosurgeryThomas Jefferson University HospitalThomas Jefferson University Hospital

Spinal Cord AnatomySpinal Cord Anatomy

31 pairs of spinal nerves31 pairs of spinal nerves8 cervical8 cervical12 thoracic12 thoracic5 lumbar5 lumbar5 sacral5 sacral1 1 coccygealcoccygeal

C & L enlargementsC & L enlargementsConusConus tapers to ~L1/2tapers to ~L1/2FilumFilum terminaleterminale-- attaches to attaches to dorsum of 1dorsum of 1stst coccygealcoccygealvertebravertebra

Spinal Cord AnatomySpinal Cord Anatomy

Central gray matterCentral gray matterNeuronal cell bodiesNeuronal cell bodiesSupporting structuresSupporting structures

Prominent ventral and Prominent ventral and dorsal components with dorsal components with commisurecommisure between halvesbetween halvesWhite matter tracts White matter tracts encircle gray matterencircle gray matter

AnatomyAnatomy--MeningesMeninges

DuraDura: closest to VB: closest to VBsingle layer, contrast with single layer, contrast with brainbrain

Delicate Delicate ArachnoidArachnoidPiaPia: contacts cord : contacts cord

attaches cord to attaches cord to duradura via via dentate ligaments.dentate ligaments.

AnatomyAnatomy--Spinal Cord VasculatureSpinal Cord Vasculature

VertVert a. gives rise to:a. gives rise to:1 Ant spinal a. &1 Ant spinal a. &2 Post Spinal a. 2 Post Spinal a.

Lower 1/3 of CLower 1/3 of C--sp sp radicularradicular aaaa. off. off

VertVert a.a.Ascending cervical a.Ascending cervical a.Deep cervical a.Deep cervical a.


Below CBelow C--spine spine continuous continuous anastomosesanastomoseswith with radicularradicular arteriesarteriesAortaAorta intercostalintercostal a.a.spinal a.spinal a. ant & post ant & post radicularradicular a.a.Central branches off Central branches off ASA alternate sides of ASA alternate sides of cordcord


Artery of Artery of AdamkiewiczAdamkiewiczLeft T11 (T9Left T11 (T9--12) 12) radicularradicularart.art.Major blood supply to Major blood supply to lower T and L spinelower T and L spine


Batson’s plexus: Batson’s plexus: epidural veinsepidural veinsno valvesno valves

Multiple Multiple anastamosesanastamoses w/w/AzygousAzygous systemsystemIVCIVCPelvic plexusPelvic plexusProstaticProstatic plexusplexus

HistoryHistory

Sir Victor Horsley (1857Sir Victor Horsley (1857--1916)1916)1887: 11887: 1stst successful resection successful resection of of intraduralintradural spinal neoplasmspinal neoplasm

MeningiomaMeningioma

1911: 11911: 1stst successful resection of successful resection of intramedullaryintramedullary tumortumor

Charles Charles ElsbergElsberg2 stage procedure 2 stage procedure myelotomymyelotomy, , 1wk later remove extruded 1wk later remove extruded tumortumor

Classification: Classification: IntraduralIntradural

ExtramedullaryExtramedullary: ~90%: ~90%in in subarachnoidsubarachnoid spacespaceSchwannomaSchwannomaNeurofibromaNeurofibromaMeningiomaMeningioma

>90% nerve sheath tumor >90% nerve sheath tumor or or meningiomameningioma

SubarachnoidSubarachnoid metsmets (only (only 4% of spinal 4% of spinal metsmets) or ) or “drop “drop metsmets””PedsPeds: : DermoidDermoid/ / EpidermoidEpidermoid

IntramedullaryIntramedullary: ~10%: ~10%within spinal cordwithin spinal cordEpendymomaEpendymomaAstrocytomaAstrocytomaHemangioblastomaHemangioblastomaMets (only 2% of spinal Mets (only 2% of spinal metsmets))

ExtramedullaryExtramedullary: Nerve Sheath : Nerve Sheath TumorsTumors

SchwannomasSchwannomasTogether ~1/3 of Together ~1/3 of intraduralintradural neoplasmsneoplasmsSlightly more commonSlightly more commonDorsal rootDorsal rootNeurofibromatosis (NFNeurofibromatosis (NF--2)2)EncapsulatedEncapsulatedDisplace nerveDisplace nerveSchwannSchwann cellscellsMalignancy v. rareMalignancy v. rare

NeurofibromasNeurofibromasTogether ~1/3 of Together ~1/3 of intraduralintraduralneoplasmsneoplasmsSlightly less commonSlightly less commonDorsal rootDorsal rootNeurofibromatosis (NFNeurofibromatosis (NF--1)1)UnencapsulatedUnencapsulatedEntangle nerveEntangle nerve-- elongateelongateSchwannSchwann cells & fibroblastscells & fibroblasts55--10% pts w/ NF malignant 10% pts w/ NF malignant NST (NST (≤≤ 1 yr survival)1 yr survival)

XRT implicatedXRT implicatedUsually Usually plexiformplexiform

Nerve Sheath TumorsNerve Sheath Tumors

Extended growth period Extended growth period osseous remodelingosseous remodelingWidened neural foraminaWidened neural foraminaVB scallopingVB scallopingIncreased Increased intrapedicularintrapediculardistancedistanceDumbbell shape (may have Dumbbell shape (may have extraduralextradural component up to component up to 15%)15%)

Nerve Sheath TumorNerve Sheath Tumor

T1WI: T1WI: isoiso/hypo/hypo--intenseintenseT2WI: T2WI: hyperintensehyperintenseincreased water contentincreased water contentHomogeneous enhancementHomogeneous enhancement““Target signTarget sign””: T2 or T1 with : T2 or T1 with gadgad

hyperintensehyperintense rim, hypo centerrim, hypo centerNeurofibromasNeurofibromas w/ peripheral w/ peripheral myxomatousmyxomatous & central & central fibrocollagenousfibrocollagenous tissuetissue

40% 40% schwannomasschwannomas cysticcystic

Clinical Presentation: NSTClinical Presentation: NST

Middle aged adults (Middle aged adults (male~femalemale~female prevalence)prevalence)Uniform distribution in spine Uniform distribution in spine Symptoms similar to HNPSymptoms similar to HNP

Pain and Pain and radiculopathiesradiculopathiesParesthesiasParesthesiasWeaknessWeaknessMyelopathyMyelopathy

Nerve Sheath TumorNerve Sheath Tumor--SchwannomaSchwannoma

31 nerve roots sacrificed31 nerve roots sacrificedC5C5--T1 (n=14)T1 (n=14)L3L3--S1 (n=17)S1 (n=17)

23% w/ post23% w/ post--op motor or sensory deficit (7/31)op motor or sensory deficit (7/31)6 cases 6 cases neurofibromaneurofibroma like characteristicslike characteristics

No deficitNo deficit

“Spinal roots giving rise to “Spinal roots giving rise to schwannomasschwannomas are are frequently nonfunctional at the time of surgery.”frequently nonfunctional at the time of surgery.”

Kim et al., J. Neurosurgery, 1989

Spinal Spinal MeningiomaMeningioma

2525--30% of spinal tumors30% of spinal tumors1:8 spinal to intracranial1:8 spinal to intracranialMost dorsal or lateral to Most dorsal or lateral to cordcordSolitary (only 1Solitary (only 1--2% 2% multiple)multiple)≤ 5% ≤ 5% extraduralextradural or bothor both


80% in T80% in T--spine (15% Cspine (15% C--spine)spine)Rare bone remodelingRare bone remodelingIsointenseIsointense to cord T1 & T2, to cord T1 & T2, bright homogeneous bright homogeneous enhancementenhancement-- ““duraldural tail”tail”

Clinical Presentation: Spinal Clinical Presentation: Spinal MeningiomaMeningioma

MiddleMiddle--aged women (80% women)aged women (80% women)Motor deficit: 90%Motor deficit: 90%Sensory deficit: 60%Sensory deficit: 60%PainPain: 50: 50--70% (diffuse localized over region or 70% (diffuse localized over region or radicularradicular))Sphincter dysfunctionSphincter dysfunction--~50%~50%


N=174 (143 women, 31 men)N=174 (143 women, 31 men)96.5% Gross total resection96.5% Gross total resectionSurgical mortality Surgical mortality 1%1%Recurrence rate Recurrence rate 6%6%92% good92% good--excellent postexcellent post--op op neuroneuro status status

longlong--term followterm follow--up (avg. 15 yrs) up (avg. 15 yrs) 70% pre70% pre--opop

Even Even anteriorlyanteriorly positioned tumors were positioned tumors were resectedresectedvia posterior approach (sectioning dentate via posterior approach (sectioning dentate liglig.).)

Solero et al., Neurosurgery, 1989

IntramedullaryIntramedullary NeoplasmsNeoplasms

2% of adult & 10% of pediatric CNS 2% of adult & 10% of pediatric CNS neoplasmsneoplasmsAdults Adults 5050--70% 70% EpendymomasEpendymomasPedsPeds 5555--65% 65% AstrocytomasAstrocytomasHemangioblastomasHemangioblastomas 5%5%Miscellaneous Miscellaneous 5% 5%

((gangliogliomasgangliogliomas, , oligodendrogliomasoligodendrogliomas, , paragangliomasparagangliomas))

Mets Mets v. rare (2% of spinal v. rare (2% of spinal metsmets))

IntramedullaryIntramedullary GlialGlial NeoplasmsNeoplasms

EpendymomaEpendymomaCellular (CCellular (C--sp or anywhere;)sp or anywhere;)MyxopapillaryMyxopapillary ((conusconus; slight ; slight ♂)♂)Mean age: 43 Mean age: 43 y/oy/oCystic degeneration (>50%) Cystic degeneration (>50%) w/ hemorrhage at marginsw/ hemorrhage at marginsDiffuse cord enlargement Diffuse cord enlargement multiple levelsmultiple levelsSharp Sharp deliniationdeliniation from cord from cord good planegood planeHomogeneous Homogeneous enhancementenhancement

AstrocytomaAstrocytomaLowLow--grade: grade: fibrillaryfibrillaryAA & GBM (10% AA & GBM (10% pedspeds & & 20% adults)20% adults)Mean age: 21 Mean age: 21 y/oy/oCystic as well, less likely to Cystic as well, less likely to hemorrhagehemorrhageDiffuse cord enlargement Diffuse cord enlargement multiple levelsmultiple levelsMore infiltrative often poor More infiltrative often poor planeplaneHeterogeneous Heterogeneous enhancementenhancement

IntramedullaryIntramedullary EpendymomaEpendymoma

19-year-old presented w/ numbness and finger clumsiness. a. Coronal T1WI demonstrates a rostral cyst & expansile cervical tumor.b. Sagittal T1WI demonstrates the enhancing tumor from C2–C5. c. The axial T1WI w/ gad: characteristic central location of this tumor type

IntramedullaryIntramedullary AstrocytomaAstrocytoma

IntramedullaryIntramedullary GlialGlial NeoplasmsNeoplasms: : Clinical PresentationClinical Presentation

Pain present over extended timePain present over extended timeOften localized to spinal segmentOften localized to spinal segmentWorse @ night/awakening Worse @ night/awakening hypercarbiahypercarbia venous venous engorgementengorgement

Gait abnormalities (spastic paresis or ataxia)Gait abnormalities (spastic paresis or ataxia)Sensory changesSensory changesHighHigh--grade grade astrocytomasastrocytomas sxssxs for mean of 4for mean of 4--7 7 mosmos vsvs lowlow--grade mean grade mean sxsx duration 41 mos.duration 41 mos.

IntramedullaryIntramedullary GlialGlial NeoplasmsNeoplasms

>90% 5>90% 5--yr survivalyr survivalGoal: Goal: bxbx & prevent & prevent further further neuroneuro deficitdeficit

N=239 lowN=239 low--grade spinal grade spinal neoplasmsneoplasmsNeurologicNeurologic outcomeoutcome

40% improved40% improved50% unchanged50% unchanged10% worsened10% worsened

Brotchi et al., Contemp Neurosurg., 1999

IntramedullaryIntramedullary GlialGlial NeoplasmsNeoplasmsN=69 (N=69 (intramedullaryintramedullary spinal cord tumors)spinal cord tumors)NeuroNeuro outcome (mean outcome (mean f/uf/u of 54 mos.)of 54 mos.)

20% improved20% improved50% unchanged50% unchanged30% worsened 30% worsened Improvement @ 6Improvement @ 6--18 18 mosmos ((dosaldosal columns longest)columns longest)PreopPreop neuroneuro fxnfxn best prognostic indicator outcomebest prognostic indicator outcome

5/6 high grade 5/6 high grade astrocytomasastrocytomas died by 9died by 9--16 16 mosmos1 alive @ 10 1 alive @ 10 mosmos but with progressionbut with progression

Cristante & Hermann, Neurosurg, 1994

IntramedullaryIntramedullary EpendymomaEpendymoma

N=23, N=23, intramedullaryintramedullary ependymomaependymoma8 8 reoperationreoperation; only 4 ; only 4 conusconus, 0 , 0 filumfilumGTR in all casesGTR in all cases

Mean Mean f/uf/u of 62 of 62 mosmos (6 mos.(6 mos.--13yrs)13yrs)No pts lost to No pts lost to f/uf/uNo recurrenceNo recurrence8 pts improved8 pts improved12 pts unchanged12 pts unchanged3 pts deteriorated3 pts deteriorated

With GTR no role for adjuvant With GTR no role for adjuvant TxTx

McCormick et al., J of Neurosurg, 1990

IntramedullaryIntramedullary AstrocytomaAstrocytoma

N=25 N=25 intramedullaryintramedullary astrocytomasastrocytomas6 pts with high6 pts with high--grade grade 5 died (45 died (4--23 23 mosmos postpost--op)op)2 pts with advanced 2 pts with advanced neuroneuro disability disability preoppreop died from died from medical complicationsmedical complications

17 pts w/ mean 17 pts w/ mean f/uf/u of 50 mos. (16of 50 mos. (16--89 89 mosmos))FxnFxn: 3 pts improved, 12 : 3 pts improved, 12 unchgedunchged, 2 worse, 2 worse15 pts: no tumor recurrence15 pts: no tumor recurrence2 pts: small residual neoplasm without progression2 pts: small residual neoplasm without progression

Surgery beneficial in lowSurgery beneficial in low--grade but not AAgrade but not AA

Epstein et al., J. Neurosurgery, 1992

Adjuvant TherapyAdjuvant Therapy

EpendymomaEpendymomaFollow w/ serial MRI if Follow w/ serial MRI if GTRGTRLocal XRT ~50 Local XRT ~50 GyGy if if subtotal subtotal resexnresexn or or disseminated disseminated dzdzNo role for chemoNo role for chemo

AstrocytomaAstrocytomaFollow w/ serial MRI if Follow w/ serial MRI if GTR, lowGTR, low--grade & wellgrade & well--circumscribedcircumscribedIf highIf high--grade of diffuse:grade of diffuse:50 50 GyGy local XRT in 30 local XRT in 30 fractionsfractionsChemo: Chemo: TemozolomideTemozolomideor PCV (or PCV (procarbozineprocarbozine/ / CCNU/ CCNU/ vincristinevincristine))

Stereotactic spinal radiosurgery yet to be defined

IntramedullaryIntramedullary HemangioblastomaHemangioblastoma

~1/3 of pts with VHL~1/3 of pts with VHL80% 80% syptomaticsyptomatic by 5by 5thth

decadedecadePresentation similar to Presentation similar to glialglial neoplasmneoplasm

Rarely present w/ sudden Rarely present w/ sudden deficit from hemorrhagedeficit from hemorrhage

IntramedullaryIntramedullary HemangioblastomaHemangioblastoma

Bright homogeneous Bright homogeneous enhancementenhancementNo more than 1 VB in No more than 1 VB in lengthlength80% w/ cystic tumor 80% w/ cystic tumor nodule; nodule; serpiginousserpiginous vesselsvesselsAA--gram & gram & emboembo possible possible prior to surgeryprior to surgery

Thank YouThank You

MRI in a 19-year-old male who presented with numbness and finger clumsiness. Histological diagnosis was an ependymoma.

a. Coronal T1-weighted MRI demonstrates a rostral cyst and expansile cervical tumor.b. Sagittal T1-weighted MRI demonstrates the enhancing tumor from C2–C5. c. The axial T1-weighted images with contrast demonstrate the characteristic central

location of this tumor type

Figure 1A and B. (A) MRI of the cervical spine, performed first, shows an area of irregular enhancement within the cord at C2-3, with an associated multiloculated syrinx extending in both cranial and caudal directions. The very intense enhancement of the lesion marks it as a hemangioblastoma and prompted a spinal survey. (B) MRI of the thoracic spine shows a second lesion at T9-10, containing a flow void and also showing bright enhancement. Cystic change within the cord extends all the way from the cervical lesion to the thoracic tumor.

HemangioblastomaHemangioblastoma

Figure 1C. The intraoperativeappearance of the cervical cord gives a classic picture of hemangioblastoma in situ, with engorged, numerous arteries and draining veins leading to and from a well-circumscribed and highly vascular tumor visible at the pialsurface. This tumor, as well as its thoracic counterpart, was excised completely and its suspected identity confirmed. She remains well (with asymptomatic pancreatic cysts) six years after diagnosis.

FIGURE 1.FIGURE 1. Schematic Schematic drawingdrawing (A(A) and ) and representative T2representative T2--weighted weighted sagittalsagittal magnetic resonance magnetic resonance imageimage (B(B) demonstrate a ) demonstrate a strictly strictly intraduralintradural tumor tumor (Group 1 tumor).(Group 1 tumor).From:From: Jinnai: Jinnai: Neurosurgery, Volume Neurosurgery, Volume 56(3).March 2005. 51056(3).March 2005. 510--515.515.

FIGURE 2.FIGURE 2. Schematic Schematic drawingdrawing (A(A) and ) and representative gadoliniumrepresentative gadolinium--enhanced T1enhanced T1--weighted coronal weighted coronal magnetic resonance imagemagnetic resonance image (B(B) ) demonstrate a tumor with demonstrate a tumor with both both intraduralintradural and and extraduralextradural components within components within the spinal canal (Group 2 the spinal canal (Group 2 tumor).tumor).

FIGURE 3.FIGURE 3. Schematic drawingSchematic drawing(A(A) and representative gadolinium) and representative gadolinium--enhanced T1enhanced T1--weighted coronal weighted coronal magnetic resonance imagemagnetic resonance image (B(B) ) demonstrate a strictly demonstrate a strictly extraduralextraduraltumor within the spinal canal tumor within the spinal canal (Group 3 tumor).(Group 3 tumor).

From:From: Jinnai: Neurosurgery, Jinnai: Neurosurgery, Volume 56(3).March Volume 56(3).March 2005.5102005.510--515515

FIGURE 4.FIGURE 4. Schematic Schematic drawingdrawing (A(A) and ) and representative gadoliniumrepresentative gadolinium--enhanced T1enhanced T1--weighted axialweighted axial(B(B) and coronal) and coronal (C(C) magnetic ) magnetic resonance images demonstrate resonance images demonstrate a strictly a strictly extraduralextradural tumor tumor extending through the extending through the intervertebralintervertebral foramen foramen (Group 4 tumor).(Group 4 tumor).

FIGURE 5.FIGURE 5. Schematic drawingSchematic drawing(A(A) and representative gadolinium) and representative gadolinium--enhanced T1enhanced T1--weighted coronal weighted coronal magnetic resonance imagemagnetic resonance image (B(B) ) demonstrate a tumor with both demonstrate a tumor with both intraduralintradural and and extraduralextraduralcomponents extending through the components extending through the intervertebralintervertebral foramen (Group 5 foramen (Group 5 tumor).tumor). Arrow Arrow indicates an indicates an intraduralintradural component.component.

Bar graph showing classification of spinal nerve sheath tumors at the various spinal levels.


VertVert a. gives rise to 1 a. gives rise to 1 Ant spinal a. & 2 Post Ant spinal a. & 2 Post Spinal a. (see JSH Spinal a. (see JSH anatomy talk)anatomy talk)Blood from Blood from vertsvertssupply cervical spine, supply cervical spine, but below is but below is continuous continuous anastomosesanastomoses with with radicularradicular arteriesarteries

IntraduralIntradural--ExtramedullaryExtramedullary

Nerve Sheath Tumors:Nerve Sheath Tumors:SchwannomasSchwannomas slightly more common than slightly more common than

NeurofibromasNeurofibromasDorsal root (sensory)Dorsal root (sensory)3535--45% have Neurofibromatosis: 45% have Neurofibromatosis: NeurofibromasNeurofibromasw/ NFw/ NF--1 & 1 & SchwannomasSchwannomas w/ NFw/ NF--2 (p676 Wilkins)2 (p676 Wilkins)SchwannSchwann cells vs. cells vs. SchwannSchwann cells & fibroblastscells & fibroblastsDisplace nerve vs. Entangle nerve fasciclesDisplace nerve vs. Entangle nerve fasciclesMalignant nerve sheath tumor degeneration: Malignant nerve sheath tumor degeneration: increased incidence with NFincreased incidence with NF

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