TherapyofMalignantPheochromocytoma

惡性嗜鉻細(xì)胞瘤的治療LiteratureReport惡性嗜鉻細(xì)胞瘤的治療Introduction

ruleof10sforpheochromocytoma(PCC)

10%bilateral10%extra-adrenal10%extra-abdomen10%malignant10%familial10%children10%normalbloodpressure2025/6/232惡性嗜鉻細(xì)胞瘤的治療IntroductionThemostfrequentsiteofmetastasesistheskeletonAdditionalsitesareliver,retroperitoneumwithlymphnodes,CNS,pleura,andkidney2025/6/233惡性嗜鉻細(xì)胞瘤的治療Malignantvs.BenignCurrently,thereisnoeffectivecureformalignantpheochromocytoma.Therearealsonoreliablehistopathologicalmethodsfordistinguishingbenignfrommalignanttumors.Malignancyrequiresevidenceofmetastasesatnon-chromaffinsitesdistantfromthatoftheprimarytumor.2025/6/234惡性嗜鉻細(xì)胞瘤的治療Metastaticdiseaseinpheochromocytomamaybepresentatthetimeofinitialdiagnosisormayonlybecameevidentaftersurgicalremovaloftheprimarytumor,usuallywithin5years,butsometimes16ormoreyearslater.2025/6/235惡性嗜鉻細(xì)胞瘤的治療Duetotherarityofthetumor,clinicalstudiesaboutpheochromocytomasufferfromafragmentednatureandusuallyinvolvetoosmallanumberofcasestoreachconclusiveresults.2025/6/236惡性嗜鉻細(xì)胞瘤的治療Becausethereiscurrentlynoeffectivecureformalignantpheochromocytoma,mosttreatmentarepalliative,butinsomecasesmayreducetumorburdenandprolongsurvival.Withouttreatment,the5-yearsurvivalisgenerallylessthan50%.Thecourse,however,canbehighlyvariablewithoccasionalpatientslivingmorethan20yearsafterdiagnosis.2025/6/237惡性嗜鉻細(xì)胞瘤的治療Oncemalignancyisdiagnosed,therapyisgenerallydirectedatcontrollingbloodpressure,butmayalsoincludetumordebulking.2025/6/238惡性嗜鉻細(xì)胞瘤的治療AlternativeofCurrentTherapySurgeryRadiopharmaceuticalsCombinedChemotherapyArterialEmbolization2025/6/239惡性嗜鉻細(xì)胞瘤的治療AlternativeofCurrentTherapySurgeryRadiopharmaceuticalsCombinedChemotherapyArterialEmbolization2025/6/2310惡性嗜鉻細(xì)胞瘤的治療PrimarysurgicalresectionisthetreatmentofchoicewheneverpossibleLimiteddisease:curativeintentionExtendeddisease:stilltobeconsideredinthefirstplacefordebulkingandaspalliativetreatment(Mundschenketal.1998)2025/6/2311惡性嗜鉻細(xì)胞瘤的治療ProblemWhensignsofregionalinvolvementordistantdiseaseareabsent,thereiscurrentlynoreliablepreoperativediagnostictestthatcandifferentiatebetweenmalignantandbenignpheochromocytomasShouldpheochromocytomasizeinfluencesurgicalapproach?2025/6/2312惡性嗜鉻細(xì)胞瘤的治療Acomparisonof90malignantand60benignpheochromocytomas

(WenT.Shenetal.2004)ComparisonoftumorsizeforbenignpheochromocytomasandmalignantpheochromocytomaswithlocaldiseaseonlySizedoesnotreliablypredictmalignancyinpheochromocytomaswithlocaldiseaseonly2025/6/2313惡性嗜鉻細(xì)胞瘤的治療Malignant(n

=

29)Benign(n

=

55)Tumorsize(mean±SD)6.1±3.1cm5.3±2.3cm<2cm012.0-3.9cm9104.0-5.9cm6256.0-7.9cm5138.0-9.9cm53≥10cm432025/6/2314惡性嗜鉻細(xì)胞瘤的治療MalignantPCCspresentingwithonlylocaldiseasecannotbediscriminatedfrombenignPCCsbysizealone.WhenPCCsdonothaveevidenceofinvasionordistantmetastasesandthesurgeonacquiresanappropriatelevelofexperience,themajorityofthesetumorscanbesafelyresectedlaparoscopically.2025/6/2315惡性嗜鉻細(xì)胞瘤的治療Laparoscopicadrenalectomyforpheochromocytomashouldbeconvertedtoopenadrenalectomyfordifficultdissection,invasion,adhesions,orsurgeoninexperience2025/6/2316惡性嗜鉻細(xì)胞瘤的治療SurgicalapproachTransabdominalapproachisnecessaryminimallyinvasiveproceduresretroperitonealapproachesshouldbeabandonedtodefinitelypreservethetumorcapsuleandperformtotallymphadecectomy(Orchardetal.1993)2025/6/2317惡性嗜鉻細(xì)胞瘤的治療SecondaryTumorsNoexperiencewithadjuvantpreandpostoperativeradiationexistsGenerallyaremultipleRadicalsurgicalresectionisoftenimpossibleOthertreatmentmodalitieshavetobeconsidered2025/6/2318惡性嗜鉻細(xì)胞瘤的治療AlternativeofCurrentTherapySurgeryRadiopharmaceuticalsCombinedChemotherapyArterialEmbolization2025/6/2319惡性嗜鉻細(xì)胞瘤的治療2025/6/2320惡性嗜鉻細(xì)胞瘤的治療131I-MIBGisthetreatmentofchoiceforallunresectable,MIBGpositivetumors58casesofmalignantPCCtreatedby131I-MIBG—therapeuticresultsandadverseevents(ZHURuisenetal.1999)2025/6/2321惡性嗜鉻細(xì)胞瘤的治療Patientswereclassifiedinto3groupsaccordingtotheirtumorsize<8cm3(11cases),8～20cm3(21cases),>20cm3(26cases)Ingroup1,themeanabsorptiondosepergramoftumorwasabove1000cGy.Aftertreatment,tumorsdisappearedorshrinkedinallpatients2025/6/2322惡性嗜鉻細(xì)胞瘤的治療Ingroup2,theabsorptiondosewassimilartothatofgroup1,butthemeanabsorptiondosepergramwas717.6cGy,andtumormassregressionwas36%;76%reducedurinarycatecholamineIngroup3,theabsorptiondosepergramtumortissuewas277cGy,and30%tumorenlargement,20%died;theremaining50%symptomaticimprovementwithoutanychangeintumorsize2025/6/2323惡性嗜鉻細(xì)胞瘤的治療131I-MIBGisofcertaintherapeuticeffectivenessofsymptomaticimprovementCompletetumormassdisappearancehasonlybeenfoundinsmalltumorsTreatmentwith131I-MIBGshouldbeinstitutedimmediatelyaftersurgicalresectiontoeradicatetheresidualtumorcellsandtopreventrecurrencesBonemarrowsuppressionistemporaryandnotdosagerelated2025/6/2324惡性嗜鉻細(xì)胞瘤的治療In1997,Lohetal.publishedareviewoftheworldwideexperienceinvolving116patientstreatedwith131I-MIBGformalignantpheochromocytoma.Overall,therewasasymptomaticresponsein76%,ahormonalresponsein45%,andtumorregressionin30%.Theactivityof131I-MIBGpersingledosewas96–300mCi,andthemeancumulativeactivitywas490±350mCi.OnlyfiveCRsto131I-MIBGwerereported.2025/6/2325惡性嗜鉻細(xì)胞瘤的治療LimitationsNotallpatientswithmultiplemetastasesofmalignantpheochromocytomashavesufficientuptakeofMIBGtoallowMIBGtherapyMIBGnegativelesionscoexistwithMIBGpostivelesions,requiringcombinedtreatment2025/6/2326惡性嗜鉻細(xì)胞瘤的治療Asasingleagent,131I-MIBGhaslimitedefficacyintreatingmalignantpheochromocytoma.Itsuseincombinationwithothercytotoxicagents,asiscurrentlybeingstudiedinpatientswithneuroblastoma,mayresultinadditionalbenefit(Sissonetal.1999)2025/6/2327惡性嗜鉻細(xì)胞瘤的治療AlternativeofCurrentTherapySurgeryRadiopharmaceuticalsCombinedChemotherapyArterialEmbolization2025/6/2328惡性嗜鉻細(xì)胞瘤的治療Onlysparsedataonchemotherapeuticregimensareavailable,mostoftheminreportsoffewcasesThemostwell-establishedregimenisCVD(Averbuchetal.1988)CTX750mg/m2d1,VCR1.4mg/m2d1,Dacarbazine600mg/m2d1,2Cycle21days2025/6/2329惡性嗜鉻細(xì)胞瘤的治療TheCVDregimenwasbasedonthetreatmentforadvancedneuroblastoma.Thisregimenhasbeenreportedtoproducegoodresponsesinmalignantpheochromocytoma,butthemediandurationofremissionis21monthsCompletelong-termdiseaseremissionswithchemotherapyhavenotbeenreported.2025/6/2330惡性嗜鉻細(xì)胞瘤的治療AlternativeofCurrentTherapySurgeryRadiopharmaceuti