Spinal Cord Tumors د.عارف

8/3/2019 Spinal Cord Tumors .

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IntraduralIntradural SpinalSpinalNeoplasmsNeoplasms

John K.John K. BirknesBirknes, M.D., M.D.

Department of NeurosurgeryDepartment of Neurosurgery

Thomas Jefferson University HospitalThomas Jefferson University Hospital


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Spinal Cord AnatomySpinal Cord Anatomy

31 pairs of spinal nerves31 pairs of spinal nerves

8 cervical8 cervical 12 thoracic12 thoracic

5 lumbar5 lumbar

5 sacral5 sacral

11 coccygealcoccygeal

C & L enlargementsC & L enlargements

ConusConus tapers to ~L1/2tapers to ~L1/2

FilumFilum terminaleterminale-- attaches toattaches todorsum of 1dorsum of 1stst coccygealcoccygeal

vertebravertebra


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Spinal Cord AnatomySpinal Cord Anatomy

Central gray matterCentral gray matter

Neuronal cell bodiesNeuronal cell bodies

Supporting structuresSupporting structures

Prominent ventral andProminent ventral and

dorsal components withdorsal components withcommisurecommisure between halvesbetween halves

White matter tractsWhite matter tracts

encircle gray matterencircle gray matter


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AnatomyAnatomy--MeningesMeninges

DuraDura: closest to VB: closest to VB

single layer, contrast withsingle layer, contrast withbrainbrain

DelicateDelicateArachnoidArachnoid

PiaPia: contacts cord: contacts cord attaches cord toattaches cord to duraduraviavia

dentate ligaments.dentate ligaments.


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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--spsp radicularradicular aaaa. off. off

VertVert a.a.

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


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Below CBelow C--spinespine

continuouscontinuous anastomosesanastomoseswithwith radicularradicular arteriesarteries

AortaAorta intercostalintercostal a.a.

spinal a.spinal a. ant & postant & postradicularradicular a.a.

Central branches offCentral branches off

ASA alternate sides ofASA alternate sides ofcordcord


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Artery ofArtery ofAdamkiewiczAdamkiewicz

Left T11 (T9Left T11 (T9--12)12) radicularradicularart.art.

Major blood supply toMajor blood supply to

lower T and L spinelower T and L spine


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Batsons plexus:Batsons plexus:

epidural veinsepidural veins

no valvesno valves

MultipleMultiple anastamosesanastamosesw/w/ AzygousAzygous systemsystem

IVCIVC

Pelvic plexusPelvic plexus ProstaticProstatic plexusplexus


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HistoryHistory

Sir Victor Horsley (1857Sir Victor Horsley (1857--1916)1916)

1887: 11887: 1stst successful resectionsuccessful resectionofofintraduralintradural spinal neoplasmspinal neoplasm

MeningiomaMeningioma

1911: 11911: 1stst successful resection ofsuccessful resection ofintramedullaryintramedullarytumortumor

CharlesCharles ElsbergElsberg

2 stage procedure2 stage procedure myelotomymyelotomy,,1wk later remove extruded1wk later remove extruded

tumortumor


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Classification:Classification: IntraduralIntradural

ExtramedullaryExtramedullary: ~90%: ~90%

inin subarachnoidsubarachnoid spacespace SchwannomaSchwannoma

NeurofibromaNeurofibroma

MeningiomaMeningioma >90% nerve sheath tumor>90% nerve sheath tumor

oror meningiomameningioma

SubarachnoidSubarachnoid metsmets (only(only4% of spinal4% of spinal metsmets) or) ordropdrop metsmets

PedsPeds:: DermoidDermoid//EpidermoidEpidermoid

IntramedullaryIntramedullary: ~10%: ~10%

within spinal cordwithin spinal cord EpendymomaEpendymoma

AstrocytomaAstrocytoma

HemangioblastomaHemangioblastoma

Mets (only 2% of spinalMets (only 2% of spinalmetsmets))


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ExtramedullaryExtramedullary: Nerve Sheath: Nerve Sheath

TumorsTumors

SchwannomasSchwannomas

Together ~1/3 ofTogether ~1/3 ofintraduralintradural neoplasmsneoplasms

Slightly more commonSlightly more common

Dorsal rootDorsal root

Neurofibromatosis (NFNeurofibromatosis (NF--2)2) EncapsulatedEncapsulated

Displace nerveDisplace nerve

SchwannSchwann cellscells Malignancy v. rareMalignancy v. rare

NeurofibromasNeurofibromas

Together ~1/3 ofTogether ~1/3 ofintraduralintraduralneoplasmsneoplasms

Slightly less commonSlightly less common

Dorsal rootDorsal root

Neurofibromatosis (NFNeurofibromatosis (NF--1)1) UnencapsulatedUnencapsulated

Entangle nerveEntangle nerve-- elongateelongate

SchwannSchwann cells & fibroblastscells & fibroblasts

55--10% pts w/ NF malignant10% pts w/ NF malignantNST (NST ( 1 yr survival)1 yr survival) XRT implicatedXRT implicated

UsuallyUsuallyplexiformplexiform


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Nerve Sheath TumorsNerve Sheath Tumors Extended growth periodExtended growth period

osseous remodelingosseous remodeling

Widened neural foraminaWidened neural foramina

VB scallopingVB scalloping

IncreasedIncreased intrapedicularintrapediculardistancedistance

Dumbbell shape (may haveDumbbell shape (may have

extraduralextradural component up tocomponent up to15%)15%)


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Nerve Sheath TumorNerve Sheath Tumor T1WI:T1WI: isoiso/hypo/hypo--intenseintense

T2WI:T2WI: hyperintensehyperintense increased water contentincreased water content

Homogeneous enhancementHomogeneous enhancement

Target signTarget sign: T2 or T1 with: T2 or T1 withgadgad hyperintensehyperintense rim, hypo centerrim, hypo center

NeurofibromasNeurofibromasw/ peripheralw/ peripheral

myxomatousmyxomatous & central& centralfibrocollagenousfibrocollagenous tissuetissue

40%40% schwannomasschwannomas cysticcystic


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Clinical Presentation: NSTClinical Presentation: NST

Middle aged adults (Middle aged adults (male~femalemale~female prevalence)prevalence)

Uniform distribution in spineUniform distribution in spine

Symptoms similar to HNPSymptoms similar to HNP

Pain andPain and radiculopathiesradiculopathies ParesthesiasParesthesias

WeaknessWeakness

MyelopathyMyelopathy


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Nerve Sheath TumorNerve Sheath Tumor--SchwannomaSchwannoma

31 nerve roots sacrificed31 nerve roots sacrificed

C5C5--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 cases6 cases neurofibromaneurofibroma like characteristicslike characteristics No deficitNo deficit

Spinal roots giving rise toSpinal roots giving rise to schwannomasschwannomas arearefrequently nonfunctional at the time of surgery.frequently nonfunctional at the time of surgery.

Kim et al., J. Neurosurgery, 1989


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SpinalSpinal MeningiomaMeningioma

2525--30% of spinal tumors30% of spinal tumors

1:8 spinal to intracranial1:8 spinal to intracranial

Most dorsal or lateral toMost dorsal or lateral to

cordcord

Solitary (only 1Solitary (only 1--2%2%

multiple)multiple)

5% 5% extraduralextradural or bothor both


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SpinalSpinal MeningiomaMeningioma

80% in T80% in T--spine (15% Cspine (15% C--spine)spine)

Rare bone remodelingRare bone remodeling

IsointenseIsointense to cord T1 & T2,to cord T1 & T2,

bright homogeneousbright homogeneous

enhancementenhancement-- duraldural tailtail


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Clinical Presentation: SpinalClinical 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 or70% (diffuse localized over region orradicularradicular))

Sphincter dysfunctionSphincter dysfunction--~50%~50%


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SpinalSpinal MeningiomaMeningioma N=174 (143 women, 31 men)N=174 (143 women, 31 men)

96.5% Gross total resection96.5% Gross total resection Surgical mortalitySurgical mortality 1%1%

Recurrence rateRecurrence rate 6%6%

92% good92% good--excellent postexcellent post--opop neuroneuro statusstatus longlong--term followterm follow--up (avg. 15 yrs)up (avg. 15 yrs)

70% pre70% pre--opop

EvenEven anteriorlyanteriorlypositioned tumors werepositioned tumors were resectedresectedvia posterior approach (sectioning dentatevia posterior approach (sectioning dentate liglig.).)

Solero et al., Neurosurgery, 1989


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IntramedullaryIntramedullary NeoplasmsNeoplasms

2% of adult & 10% of pediatric CNS2% of adult & 10% of pediatric CNS neoplasmsneoplasms

AdultsAdults 5050--70%70% EpendymomasEpendymomas

PedsPeds 5555--65%65%AstrocytomasAstrocytomas

HemangioblastomasHemangioblastomas 5%5% MiscellaneousMiscellaneous 5%5%

((gangliogliomasgangliogliomas,, oligodendrogliomasoligodendrogliomas,,

paragangliomasparagangliomas))

MetsMets v. rare (2% of spinalv. rare (2% of spinal metsmets))


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IntramedullaryIntramedullary GlialGlial NeoplasmsNeoplasms

EpendymomaEpendymoma

Cellular (CCellular (C--sp or anywhere;)sp or anywhere;)

MyxopapillaryMyxopapillary((conusconus; slight; slight))

Mean age: 43Mean age: 43 y/oy/o

Cystic degeneration (>50%)Cystic degeneration (>50%)

w/ hemorrhage at marginsw/ hemorrhage at margins Diffuse cord enlargementDiffuse cord enlargement

multiple levelsmultiple levels

SharpSharp deliniationdeliniation from cordfrom cord

good planegood plane HomogeneousHomogeneous

enhancementenhancement


LowLow--grade:grade: fibrillaryfibrillary

AA & GBM (10%AA & GBM (10% pedspeds &&20% adults)20% adults)

Mean age: 21Mean age: 21 y/oy/o

Cystic as well, less likely toCystic as well, less likely to

hemorrhagehemorrhage Diffuse cord enlargementDiffuse cord enlargement

multiple levelsmultiple levels

More infiltrative often poorMore infiltrative often poor

planeplane HeterogeneousHeterogeneous

enhancementenhancement


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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 C2C5.

c.The axial T1WI w/ gad: characteristic central location of this tumor type


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IntramedullaryIntramedullaryAstrocytomaAstrocytoma


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IntramedullaryIntramedullary GlialGlial NeoplasmsNeoplasms::

Clinical PresentationClinical Presentation

Pain present over extended timePain present over extended time

Often localized to spinal segmentOften localized to spinal segment

Worse @ night/awakeningWorse @ night/awakening hypercarbiahypercarbiavenousvenous

engorgementengorgement

Gait abnormalities (spastic paresis or ataxia)Gait abnormalities (spastic paresis or ataxia)

Sensory changesSensory changes

HighHigh--gradegrade astrocytomasastrocytomas sxssxs for mean of 4for mean of 4--77mosmosvsvs lowlow--grade meangrade mean sxsx duration 41 mos.duration 41 mos.


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IntramedullaryIntramedullary GlialGlial NeoplasmsNeoplasms >90% 5>90% 5--yr survivalyr survival

Goal:Goal: bxbx & prevent& preventfurtherfurther neuroneuro deficitdeficit

N=239 lowN=239 low--grade spinalgrade spinalneoplasmsneoplasms

NeurologicNeurologic outcomeoutcome

40% improved40% improved

50% unchanged50% unchanged

10% worsened10% worsened

Brotchi et al., Contemp Neurosurg., 1999


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IntramedullaryIntramedullary GlialGlial NeoplasmsNeoplasms N=69 (N=69 (intramedullaryintramedullaryspinal cord tumors)spinal cord tumors)

NeuroNeuro outcome (meanoutcome (mean f/uf/u of 54 mos.)of 54 mos.)

20% improved20% improved

50% unchanged50% unchanged

30% worsened30% worsened Improvement @ 6Improvement @ 6--1818 mosmos ((dosaldosal columns longest)columns longest)

PreopPreop neuroneuro fxnfxn best prognostic indicator outcomebest prognostic indicator outcome

5/6 high grade5/6 high grade astrocytomasastrocytomas died by 9died by 9--1616 mosmos

1 alive @ 101 alive @ 10 mosmos but with progressionbut with progression

Cristante & Hermann, Neurosurg, 1994


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IntramedullaryIntramedullary EpendymomaEpendymoma N=23,N=23, intramedullaryintramedullaryependymomaependymoma

88 reoperationreoperation; only 4; only 4 conusconus, 0, 0 filumfilum GTR in all casesGTR in all cases

MeanMean f/uf/u of 62of 62 mosmos (6 mos.(6 mos.--13yrs)13yrs)

No pts lost toNo pts lost to f/uf/u No recurrenceNo recurrence

8 pts improved8 pts improved

12 pts unchanged12 pts unchanged 3 pts deteriorated3 pts deteriorated

With GTR no role for adjuvantWith GTR no role for adjuvantTxTx

McCormick et al., J of Neurosurg, 1990


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IntramedullaryIntramedullaryAstrocytomaAstrocytoma N=25N=25 intramedullaryintramedullaryastrocytomasastrocytomas

6 pts with high6 pts with high--gradegrade 5 died (45 died (4--2323 mosmos postpost--op)op) 2 pts with advanced2 pts with advanced neuroneuro disabilitydisabilitypreoppreop died fromdied from

medical complicationsmedical complications

17 pts w/ mean17 pts w/ mean f/uf/u of 50 mos. (16of 50 mos. (16--8989 mosmos)) FxnFxn: 3 pts improved, 12: 3 pts improved, 12 unchgedunchged, 2 worse, 2 worse

15 pts: no tumor recurrence15 pts: no tumor recurrence

2 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


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Adjuvant TherapyAdjuvant Therapy EpendymomaEpendymoma

Follow w/ serial MRI ifFollow w/ serial MRI ifGTRGTR

Local XRT ~50Local XRT ~50 GyGy ifif

subtotalsubtotal resexnresexn oror

disseminateddisseminated dzdz

No role for chemoNo role for chemo


Follow w/ serial MRI ifFollow w/ serial MRI ifGTR, lowGTR, low--grade & wellgrade & well--

circumscribedcircumscribed

If highIf high--grade of diffuse:grade of diffuse:

5050 GyGylocal XRT in 30local XRT in 30

fractionsfractions

Chemo:Chemo: TemozolomideTemozolomide

or PCV (or PCV (procarbozineprocarbozine//CCNU/CCNU/vincristinevincristine))

Stereotactic spinal radiosurgery yet to be defined


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IntramedullaryIntramedullary HemangioblastomaHemangioblastoma

~1/3 of pts with VHL~1/3 of pts with VHL

80%80% syptomaticsyptomatic by 5by 5ththdecadedecade

Presentation similar toPresentation similar to

glialglial neoplasmneoplasm Rarely present w/ suddenRarely present w/ sudden

deficit from hemorrhagedeficit from hemorrhage


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IntramedullaryIntramedullary HemangioblastomaHemangioblastoma

Bright homogeneousBright homogeneous

enhancementenhancement No more than 1 VB inNo more than 1 VB in

lengthlength

80% w/ cystic tumor80% w/ cystic tumornodule;nodule; serpiginousserpiginousvesselsvessels

AA--gram &gram & emboembo possiblepossible

prior to surgeryprior to surgery


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Thank YouThank You


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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 C2C5.

c.The axial T1-weighted images with contrast demonstrate the characteristic centrallocation of this tumor type

Figure 1A and B (A) MRI of the cervical spine performed first shows an area of irregular


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Figure 1A and B. (A) MRI of the cervical spine, performed first, shows an area of irregularenhancement 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 asecond lesion at T9-10, containing a flow void and also showing bright enhancement. Cysticchange within the cord extends all the way from the cervical lesion to the thoracic tumor.


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HemangioblastomaHemangioblastoma

Figure 1C. The intraoperativeappearance of the cervical cord

gives a classic picture ofhemangioblastoma in situ, withengorged, numerous arteries anddraining veins leading to and froma well-circumscribed and highly

vascular tumor visible at the pialsurface. This tumor, as well as itsthoracic counterpart, was excisedcompletely and its suspectedidentity confirmed. She remains

well (with asymptomatic pancreaticcysts) six years after diagnosis.


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FIGURE 1.FIGURE 1. SchematicSchematic

drawingdrawing(A(A) and) andrepresentative T2representative T2--weightedweightedsagittalsagittalmagnetic resonancemagnetic resonanceimageimage(B(B) demonstrate a) demonstrate a

strictlystrictlyintraduralintraduraltumortumor(Group 1 tumor).(Group 1 tumor).

From:From: Jinnai:Jinnai:

Neurosurgery, VolumeNeurosurgery, Volume56(3).March 2005. 51056(3).March 2005. 510--515.515.


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drawingdrawing(A(A) and) andrepresentative gadoliniumrepresentative gadolinium--

enhanced T1enhanced T1--weighted coronalweighted coronal

magnetic resonance imagemagnetic resonance image(B(B))demonstrate a tumor withdemonstrate a tumor with

bothbothintraduralintraduralandand

extraduralextraduralcomponents withincomponents within

the spinal canal (Group 2the spinal canal (Group 2

tumor).tumor).


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FIGURE 3.FIGURE 3. Schematic drawingSchematic drawing

(A(A) and representative gadolinium) and representative gadolinium--


magnetic resonance imagemagnetic resonance image(B(B))

demonstrate a strictlydemonstrate a strictlyextraduralextradural

tumor within the spinal canaltumor within the spinal canal(Group 3 tumor).(Group 3 tumor).

From:From: Jinnai: Neurosurgery,Jinnai: Neurosurgery,

Volume 56(3).MarchVolume 56(3).March2005.5102005.510--515515


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drawingdrawing(A(A) and) andrepresentative gadoliniumrepresentative gadolinium--

enhanced T1enhanced T1--weighted axialweighted axial

(B(B) and coronal) and coronal(C(C) magnetic) magneticresonance images demonstrateresonance images demonstrate

a strictlya strictlyextraduralextraduraltumortumor

extending through theextending through the

intervertebralintervertebralforamenforamen

(Group 4 tumor).(Group 4 tumor).


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FIGURE 5.FIGURE 5. Schematic drawingSchematic drawing

(A(A) and representative gadolinium) and representative gadolinium--


magnetic resonance imagemagnetic resonance image(B(B))

demonstrate a tumor with bothdemonstrate a tumor with both

intraduralintraduralandandextraduralextraduralcomponents extending through thecomponents extending through the

intervertebralintervertebralforamen (Group 5foramen (Group 5

tumor).tumor).ArrowArrowindicates anindicates an

intraduralintraduralcomponent.component.


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Bar graph showing classification of spinal nerve sheath

tumors at the various spinal levels.


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AnatomyAnatomy

--Spinal Cord VasculatureSpinal Cord Vasculature

VertVert a. gives rise to 1a. gives rise to 1

Ant spinal a. & 2 PostAnt spinal a. & 2 PostSpinal a. (see JSHSpinal a. (see JSH

anatomy talk)anatomy talk)

Blood fromBlood fromvertsvertssupply cervical spine,supply cervical spine,

but below isbut below is

continuouscontinuousanastomosesanastomoseswithwith

radicularradicular arteriesarteries


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IntraduralIntradural--ExtramedullaryExtramedullary

Nerve Sheath Tumors:Nerve Sheath Tumors:

SchwannomasSchwannomas slightly more common thanslightly more common thanNeurofibromasNeurofibromas

Dorsal root (sensory)Dorsal root (sensory)

3535--45% have Neurofibromatosis:45% have Neurofibromatosis: NeurofibromasNeurofibromasw/ NFw/ NF--1 &1 & SchwannomasSchwannomasw/ NFw/ NF--2 (p676 Wilkins)2 (p676 Wilkins)

SchwannSchwann cells vs.cells vs. SchwannSchwann cells & fibroblastscells & fibroblasts

Displace nerve vs. Entangle nerve fasciclesDisplace nerve vs. Entangle nerve fascicles

Malignant nerve sheath tumor degeneration:Malignant nerve sheath tumor degeneration:

increased incidence with NFincreased incidence with NF

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