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Sporadic Hemangioblastoma of the Kidney a rare renal tumor 英文参考文献
Liuetal.DiagnosticPathology2012,7:49
/content/7/1/49
CASE REPORT
OpenAccess
SporadicHemangioblastomaoftheKidney:arare
renaltumor
YangLiu1,2,Xue-shanQiu1,2*andEn-HuaWang1,2
Abstract:Hemangioblastomaisabenignandmorphologicallydistinctivetumorthatcanoccursporadicallyorin
associationwithvonHippel-Lindaudiseaseinapproximately25%ofthecases,andwhichinvolvesthecentral
nervoussysteminthemajorityofthecases.Rareoccurrencesofhemangioblastomainperipheralnervesand
extraneuraltissueshavebeenreported.Thisreportdescribesonecaseofsporadicrenalhemangioblastoma
happenedina16-year-oldChinesefemalepatient,presentingwithhematuria,andlowbackpain.Histologically,the
tumorswerecircumscribed,andcomposedofsheetsoflargepolygonalcellstraversedbyarborizingthin-walled
bloodvessels.Thediagnosisofhemangioblastomawasconfirmedbynegativeimmunostainingforcytokeratin,and
positivestainingforα-inhibin,S100andneuron-specificenolase(NSE).Thisbenignneoplasmwhichcanbe
mistakenforvariousmalignanciessuchasrenalcellcarcinoma,epithelioidhemangiopericytomaandepithelioid
angiomyolipoma,deserveswiderrecognitionforitsoccurrenceasaprimaryrenaltumor.
Virtualslides:Thevirtualslide(s)forthisarticlecanbefoundhere:http://www.diagnosticpathology.diagnomx.eu/
vs/5445834246942699.
Keywords:Hemangioblastoma,VonHippel-Lindaudisease,Renalcellcarcinoma,Epithelioidhemangiopericytoma,
Epithelioidangiomyolipoma
Background
There are only a few reports on sporadic hemangioblas-
Hemangioblastoma, also known as capillary hemangio- tomaoccurringoutsidethecentralnervoussystem,includ-
blastoma, is a benign tumor of uncertain histogenesis, ing kidney [3]. We report one such case involving the
consisting of networks of small blood vessels inter- kidney,whichmightbemistakenforotherrenaltumors,in
spersed with lipid-laden stromal cells [1]. Although the particularclearcellrenalcellcarcinoma.
stromal cells are often bland-looking, they can exhibit
significantnuclearpleomorphism,mimickingcarcinoma,
or other malignancies. Hemangioblastoma occur
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