spinal cord tumors د.عارف
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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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AnatomyAnatomy--Spinal Cord VasculatureSpinal Cord Vasculature
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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AnatomyAnatomy--Spinal Cord VasculatureSpinal Cord Vasculature
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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AnatomyAnatomy--Spinal Cord VasculatureSpinal Cord Vasculature
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
AstrocytomaAstrocytoma
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
AstrocytomaAstrocytoma
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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FIGURE 2.FIGURE 2. SchematicSchematic
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--
enhanced T1enhanced T1--weighted coronalweighted coronal
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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FIGURE 4.FIGURE 4. SchematicSchematic
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--
enhanced T1enhanced T1--weighted coronalweighted coronal
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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