肾细胞癌的分类-compressed

ClassificationofRenalCarcinomaRenalUnitCK19THPCD10、RCCUEA-1RenalCarcinomaClearCellCarcinomaGranularcellcarcinomaClearCellCarcinomaChromophobeCarcinomaPapillaryCarcinomaTypeITypeIICollectingductcarcinomaOncocytomaMetanephricAdenoma、AdenofibromaUnclassifiedandunusualcarcinomaCase137yrmale.Historyofcerebellarhemangioblastoma,renaltumorandcystGross：Tumordiameter5X5X2.5cm，cutsurfaceyellowSomecystsinthetumorRiskfactorforVonHippelLindausyndromeYoungerthan50yearsMultifocalunilateralorbilateraltumorsFamilyhistoryofrenalcancerorotherVHLrelatedtumorGermlinemutationanalysisMissensemutationofVHLgeneon3p25-26DiagnosticCriteriaforVHLSyndromeNofamilyhistoryHemangioblastomaofCNSorretina+VHLassociatedextraneuraltumorsWithfamilyhistoryHemangioblastomaofCNSorretinaorVHLassociatedextraneuraltumorsCase1DiagnosisVHLassociatedclearcellrenalcellcarcinomaVHLsubtypeHBRCCPCCMutationType1++-deletion/truncationType2A+-+Tyr98His/Tyr112HisType2B+++MissenseType2C--+Leu188ValSer80LeuHB：Hemangioblastoma；RCC：Renalcellcarcinoma；PCC：PheochromocytomaPathogenesisCohenHandMcGovernFJ,NEnglJMed,2005;353:23pVHLintumorinitiationSporadicClearCellCarcinomaPathogenesisCohenHandMcGovernFJ,NEnglJMed,2005;353:23FuhrmanNuclearGrade

（4tiered）Note：ApplyinclearcellcarcinomaandpapillarycarcinomaGradeDiameterofNucleiNucleiNucleoliOtherFeatures110μmRoundAbsenceorinconspicuous215μmFinelygranularopenchromatininconspicuous×400320μmirregularlarge×100420+μmPleomorphismlargeClumpchromatin,spindlecellMultilocularCysticRenalCellCarcinomaMultilocularCysticRenalCellCarcinomaModernPathol201023,931–936Mostofthecasesshowedchromosome3pdeletion.VerygoodsurvivalHereditaryRCCHereditaryleiomyomatosisandRCCBirt-Hogg-DubésyndromeHyperparathyroidism-JawtumorsyndromeFamilialPapillaryThyroidCancerConstitutionalchromosome3translocationsHereditarypapillaryrenalcancerHereditaryleiomyomatosisandRCCLeomyomaorleomyosarcomaofskinoruterusUnilateralpapillaryrenalcarcinomaFumarateHydratase（FH）mutation1q42Birt-Hogg-DubéSyndromeFacialfibrofolliculomaandtricodiscomaLungcystwithriskofspontaneouspneumothoraxColonicpolypsRenaltumors:oncocytomaorchromophobecarcinomaFolliculin（BHD）genemutation17p11.2Birt-Hogg-DubéSyndromeHyperparathyroidism-JawTumorSyndromeAutosomaldominantParathyroidadenomas(carcinoma)、Ossifyingfibromaofthemaxillaormandible、RenalcystsorrenaltumorWilm’stumor、mixedepithelial-stromaltumor、papillarycarcinomaHPT-JT（parafibromin）mutation1q21–q32JInternMed,2003253(6):634FamilialPapillaryThyroidCancerFamilialPTCAssociatedwithRenalCellNeoplasia1q21Notc-MetassociatedHereditaryrenalpapillarycacinomaNotCowdensyndromeNotMEN2ACase245yrmaleRightkidneymassPapillaryRenalCarcinomaType1PapillaecoveredbysmallcellswithscantycytoplasmSinglelayeronthepapillarybasementmembrane.Type2HighernucleargradewitheosinophiliccytoplasmPseudostratifiednucleionpapillarycores.Type1Type2Case2DiagnosisPapillaryRenalCellCarcinoma,TypeIPrognosisofPRCCConventionalpapillaryrenalcarcinoma75%withduplicationofchromosome7,proto-oncogeneMETCohenHandMcGovernFJ,NEnglJMed,2005;353:23PapillaryRenalCancerinvolvedinHereditarySyndromesHereditarypapillaryrenalcancerHereditaryleiomyomatosisandRCCBirt-Hogg-DubésyndromeHyperparathyroidism-JawtumorsyndromeFamilialPapillaryThyroidCancerPapillaryAdenoma

Grignon

DJ,Eble

JN.Papillaryandmetanephricadenomasofthekidney.SeminDiagnPathol.1998;15:41–53.DefinitionTumorswithpapillaryortubulararchitectureoflownuleargradeand5mmindiameterorsmallerPapillaryepithelialneoplasmoflowmalignantpotentialGrade1papillarytumorsbetween0.5-2cmarestrictlydefinedascarcinomaTumorDiametersandMetastasis

atAutopsy(871patients)MaxtumordiameternM1orN11-3cm2087(3.4%)1-2.5cm1774(2.2%)1-2.0cm1542(1.3%)≤1.0cm3960GrignonandEble.SeminDiagPathol.1998;15:41ProposedDevelopmental

StepsofPRCCKLWang,etal.HumPathol200738(3):239Case333yr,femaleRightkidneymass,2X1.5X0.5cm,surfaceyellowClear-CellPapillary

RenalCellCarcinomaClear-celltubulopapillaryrenalcellcarcinomaRenalangiomyoadenomatoustumorRenalcellcarcinomawithprominentleiomyomatousproliferationClearcellrenalcellcarcinomawithdiffusecytokeratin7immunoreactivityClear-CellPapillary

RenalCellCarcinomaCytokeratin7+,carbonicanhydraseIX(CA9)+,HIF-1α+,GLUT-1+CD10-,P504S-,TFE3-AbsenceofVHLgenealterationCase425yearsoldfemaleCTrevealedamassinrightkidneyNephrectomywasperformedTFE-3RenalcarcinomasassociatedwithXp11.2translocationsPapillaryarchitecturecomprisedofclearcellsNestedarchitectureCellswithvoluminousgranulareosinophiliccytoplasmPsammomabodiesXp11TranslocationCarcinomaHeimannetal.Fusionofanovelgene,RCC17,totheTFE3gene.CancerRes2001;61:4130Arganietal.PrimaryrenalneoplasmswithASPL-TFE3genefusionofalveolarsoftpartsarcoma.AmJSurgPathol2001;159:179Arganietal.PRCC-TFE3renalcarcinomas.AmJSurgPathol2002;26:1553Case3DiagnosisRenalcarcinomasassociatedwithXp11.2translocationsMicrophthalmiaTranscriptionFactor/TFEFamilyMiTF,TFE3,TFEB,TFECHomologousDNAbindingdomainRecognizeidenticalDNAsequencesActivatetranscriptionofsimilargenesinvitroAmJSurgPathol2005t(6;11)(p21;q12)RenalCarcinomaAges6-53years(median18yr)MorphologyEpithelioidcells,predominantlyclearcytoplasmIHCMinimalcytokeratinsexpressionAllHMB45,Melan-A(+)AberrantpositiveofTFEBtranscriptionfactorDiagnosticUseofMiTF/TFE

FamilyProteinGenesIHCAssaysNeoplasmsMiTFYesMalignantmelanomaTFE3YesAlveolarsoftpartsarcomaXp11translocationcarcinomaTFEBYest(6;11)(p21;q12)RenalCarcinomaTFECNo?ChromophobeRenalCellCarcinomaLargepalecellswithprominentcellmembranesPerinuclearhalosEosinophilicvariantMucinoustubularandspindlecellcarcinoma

Tightlypacked,small,elongatedtubulesseparatedbypalemucinousstromaTheparalleltubulararraysoftenhaveaspindlecellconfigurationCuboidalorovalnulearshapesandlow-gradefeaturesPositiveforEMA,AE1/AE3,CK7,CK19,34βE12andAMACRMTSCC-Loopoma?CollectingDuctCarcinomaCollectingDuctCarcinomaArisesintheprincipalcellsofthecollectingductsofBelliniComplextubulopapillaryarchitecturePronouncedstromaldesmoplasiaComplexinteranastomosingstructuresCK19、34bE12、UEA-1+,CD10、Villin-CK19LowGradeCollectingDuctCarcinoma-Tubulocysticcarcinoma

MacLennan

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Low-gradecollectingductcarcinomaofthekidney:reportof13casesoflow-grademucinoustubulocysticrenalcarcinomaofpossiblecollectingductorigin.

Urology

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50:679-684.Amin

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Tubulocysticcarcinomaofthekidney:Clinicopathologicanalysisof29casesofadistinctiveraresubtypeofrenalcellcarcinoma.

ModPathol

2004;

17:137ATubulocysticcarcinomaTubulesandcysticstructuresofmarkedlyvariablesize,separatedbydelicatesepta.“spiderweb-like”TubulesarelinedbyasinglelayeroflowcuboidalepithelialcellswithhobnailappearanceIHCsimilartocollectingductcarcinomaRenalMedullaryCarcinomaStronglyassociatedwithsicklecellhemoglobinopathiesTypicallylocatedintherenalmedullaMayariseintheterminalcollectingductsandtheadjacentpapillaryepitheliumReticularormicrocysticgrowthpatternTubuleformationandgrowthindiffusesheetsorsolidnodulesPleomorphicnuclei,prominentnucleoli,andeosinophiliccytoplasmIHCpositiveforCEA,EMA,CK19,topoisomeraseIIaRenalMedullaryCarcinomaAcquiredCystic