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FinalDiagnosisCase904

FinalDiagnosisCapillaryHemangioma
DIAGNOSIS
Capillaryhemangioma.
DISCUSSION
Capillaryhemangiomaisabenignvascularneoplasmcommonlyinvolvingtheskin,softtissueandmucosal
membranesoftheheadandneckregion(4).Centralnervoussystem(CNS)involvementisrelatively
uncommon,butisarecognizedoccurrence.Spinalintraduralextramedullarycapillaryhemangiomasare
extremelyrare(3),Completeresectionisthetreatmentofchoiceandfollowupof10patientsdemonstratedno
clinicalrecurrenceintheneuraxis(1).However,amongsttheadditionalninecasesreportedsince2005(2,4)is
therecentcaseofrecurrentcapillaryhemangiomaofthespinalcordina48yearoldmanthatdeveloped6
monthsafterthefirstoperation.
Spinallevelcapillaryhemangiomashavebeenreportedinadultsandadolescents.Thesetumorscanoccurinthe
thoracicareaorincloserelationtotheconusmedullarisandnerverootsofthecaudaequina.Clinical
presentationandradiologicappearanceofspinalintraduralextramedullarycapillaryhemangiomasaresimilarto
thoseofmorecommonintradurallesionsofthesamelocation(2).Theselesionsareusuallyencapsulatedand
welldemarcatedfromthesurroundingparenchymaofthespinalcordandaffectednerveroots(4).Althoughthe
presenceofenlargedvasculaturemaybeausefulclue,themagneticresonanceimagingappearanceisoften
nonspecific(2).Therefore,capillaryhemangiomashouldbeincludedinthedifferentialdiagnosisofany
enhancingintraduralextramedullarymass.
SeveralpathogenicmechanismshavebeenproposedforcapillaryhemangiomasoftheCNS:a)Impaired
movementanddifferentiationofprimitivemesodermfromtheembryonicmesodermalplateduringtheearly
somiticdifferentiation(days2124ofembryogenesis)b)Vasculartumorsoftheconuscaudaregionhavebeen
definedashamartomas,implyingtheircongenitaloriginc)Abnormaldevelopmentofvascularstructureswithin
theepineuriumofthenerverootsmaybeaffected(4).
HistologicalandimmunohistochemicalfeaturesofCNScapillaryhemangiomashavebeenreportedtobesimilar
tothosearisingintheskin.TheCNSlesionsshowedalobulararchitecturewithfibroustissueseptaandhighly
cellularareasinsixcases,whereasabloodfilledcavernousspaceandfibroendothelialpapillaemimicking
papillaryendothelialhyperplasiawereseeninfourcases.Immunohistochemistrydemonstratedexpressionof
vascularendothelialgrowthfactorandglucocorticoidreceptor(1).Ourcaseshowedthetypicalhistologic
featuresofacapillaryhemangiomaandtheimmunophenotypewascharacteristicforavascularderivedtumor.
TheKi67proliferativeindexofCNScapillaryhemangiomashasbeenreportedtorangefrom2.2to12%(mean
5.6%)(1),whiletherecentlyreportedcaseofrecurrentcapillaryhemangiomahadaproliferativeindexof10%.
Althoughtheprognosticsignificanceofthehighproliferativeindexof34%inourcaseisunclear,thisfinding
indicatesthatclosefollowupofthepatientmaybewarranted.
REFERENCES
1.AbeM,MisagoN,TanakaS,MasuokaJ,TabuchiK(2005)Capillaryhemangiomaofthecentralnervous
system:acomparativestudywithlobularcapillaryhemangiomaoftheskin.Acta
Neuropathol.109(2):1518.
2.AlakandyLM,HerculesS,BalamuraliG,ReidH,HerwadkarA,HollandJP(2006)Thoracicintradural
extramedullarycapillaryhaemangioma.BrJNeurosurg.20(4):2358.
3.KasukurthiR,RayWZ,BlackburnSL,LusisEA,SantiagoP(2009)Intramedullarycapillaryhemangioma
ofthethoracicspine:casereportandreviewoftheliterature.RareTumors.1(1):e10.
http://path.upmc.edu/cases/case904/dx.html

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4.NowakDA,WidenkaDC(2001)Spinalintraduralcapillaryhaemangioma:areview.EurSpine
J.10(6):46472.
ContributedbyGulisaTurashvili,MD,PhD,SonalVarma,MD,SandipSenGupta,MD,JohnP.Rossiter,MB,
BCh,PhD

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