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ClassificationandGeneticsofRenalCellCarcinomaZheWangDepartmentofPathology,XijingHospitalFourthMilitaryMedicalUniversity
OncocytomaCourtesyofDrJean-ChristopheFournet
RenalUnitCK19THPCD10、RCCUEA-1
RenalCarcinomaClearCellCarcinomaGranularcellcarcinomaClearCellCarcinomaChromophobeCarcinomaPapillaryCarcinomaCollectingductcarcinoma TypeI TypeIIOncocytoma Metanephric Adenoma、 AdenofibromaUnclassifiedandunusualcarcinoma
Case1?37yrmale.Historyofcerebellarhemangioblastoma,renaltumorandcyst?Gross：Tumordiameter5X5X2.5cm，cutsurfaceyellow?Somecystsinthetumor
?RiskfactorforVonHippelLindausyndrome–Youngerthan50years–Multifocalunilateralorbilateraltumors–FamilyhistoryofrenalcancerorotherVHLrelatedtumor?Germlinemutationanalysis–MissensemutationofVHLgeneon3p25-26
DiagnosticCriteriaforVHLSyndrome?Nofamilyhistory–HemangioblastomaofCNSorretina+VHLassociatedextraneuraltumors?Withfamilyhistory–HemangioblastomaofCNSorretinaorVHLassociatedextraneuraltumors
Case1DiagnosisVHLassociatedclearcellrenalcellcarcinoma
HBRCCPCCMutationType1++-deletion/truncationType2A+-+Tyr98His/Tyr112HisType2B+++MissenseType2C--+Leu188ValSer80LeuVHLsubtypeHB：Hemangioblastoma；RCC：Renalcellcarcinoma；PCC：Pheochromocytoma
PathogenesisCohenHandMcGovernFJ,NEnglJMed,2005;353:23
pVHLinTumorInitiation
SporadicClearCellCarcinoma
PathogenesisCohenHandMcGovernFJ,NEnglJMed,2005;353:23
GradeDiameterofNucleiNucleiNucleoliOtherFeatures110μmRoundAbsenceorinconspicuous215μmFinelygranularopenchromatininconspicuous×400320μmirregularlarge×100420+μmPleomorphismlargeClumpchromatin,spindlecellFuhrmanNuclearGrade（4tiered）Note：Applyinclearcellcarcinomaandpapillarycarcinoma
Cancer2005;103(1):68-75.
MultilocularCysticRenalCellCarcinoma?Wellcircumscribedwithfibrouspseudocapsule?Multicysticcutsurfacewithmarkedvariationinsizeofcysts?Cystliningusuallyconsistingo
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