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TherapyofMalignantPheochromocytoma恶性嗜铬细胞瘤的治疗LiteratureReport2023/4/282Introductionruleof10sforpheochromocytoma(PCC)10%bilateral10%extra-adrenal1

0%extra-abdomen10%malignant10%familial10%children10%normalbloodpressure2023/4/283IntroductionThemostfrequentsiteofmetastasesistheskeletonAddit

ionalsitesareliver,retroperitoneumwithlymphnodes,CNS,pleura,andkidney2023/4/284Malignantvs.BenignCu

rrently,thereisnoeffectivecureformalignantpheochromocytoma.Therearealsonoreliablehistopathologicalmethodsfordisting

uishingbenignfrommalignanttumors.Malignancyrequiresevidenceofmetastasesatnon-chromaffinsitesdistantfromthatoftheprimarytumo

r.2023/4/285Metastaticdiseaseinpheochromocytomamaybepresentatthetimeofinitialdiagnosisormayonlybecameevidentaftersurg

icalremovaloftheprimarytumor,usuallywithin5years,butsometimes16ormoreyearslater.2023/4/286Duetotherarityofthetumor,clinicalstudiesa

boutpheochromocytomasufferfromafragmentednatureandusuallyinvolvetoosmallanumberofcasestoreachconclusiveresults.2023/4/287Becausethereiscurrentlynoef

fectivecureformalignantpheochromocytoma,mosttreatmentarepalliative,butinsomecasesmayreducetumorburdena

ndprolongsurvival.Withouttreatment,the5-yearsurvivalisgenerallylessthan50%.Thecourse,however,canbehighlyvariablewithocc

asionalpatientslivingmorethan20yearsafterdiagnosis.2023/4/288Oncemalignancyisdiagnosed,therapyisgenerally

directedatcontrollingbloodpressure,butmayalsoincludetumordebulking.2023/4/289AlternativeofCurrentTherapySurgeryRadiopharmaceuticalsC

ombinedChemotherapyArterialEmbolization2023/4/2810AlternativeofCurrentTherapySurgeryRadiopharmaceuticalsComb

inedChemotherapyArterialEmbolization2023/4/2811Primarysurgicalresectionisthetreatmentofchoicewheneverpo

ssibleLimiteddisease:curativeintentionExtendeddisease:stilltobeconsideredinthefirstplacefordebulkingandaspalliativetreatment(Mundschenketa

l.1998)2023/4/2812ProblemWhensignsofregionalinvolvementordistantdiseaseareabsent,thereiscurrentlynoreliablepreoperativediagnostictes

tthatcandifferentiatebetweenmalignantandbenignpheochromocytomasShouldpheochromocytomasizeinfluencesurgicalapproach?2023/4/2813

Acomparisonof90malignantand60benignpheochromocytomas(WenT.Shenetal.2004)Comparisonoftumorsizeforbenignpheoc

hromocytomasandmalignantpheochromocytomaswithlocaldiseaseonlySizedoesnotreliablypredictmalignancyinpheochromocytomaswithlocaldiseaseonly2023/4/

2814Malignant(n=29)Benign(n=55)Tumorsize(mean±SD)6.1±3.1cm5.3±2.3cm<2cm012.0-3.9cm9104.0-5.9cm6256.0-7.9cm51

38.0-9.9cm53≥10cm432023/4/2815MalignantPCCspresentingwithonlylocaldiseasecannotbediscriminatedfrombenignPCCsbysizealone.WhenPCCsdonothave

evidenceofinvasionordistantmetastasesandthesurgeonacquiresanappropriatelevelofexperience,themajorityofthesetumorscan

besafelyresectedlaparoscopically.2023/4/2816Laparoscopicadrenalectomyforpheochromocytomashouldbeconvertedtoopenadrenalectomyfo

rdifficultdissection,invasion,adhesions,orsurgeoninexperience2023/4/2817SurgicalapproachTransabdominalapproachisnecessaryminimallyinvasiveproce

duresretroperitonealapproachesshouldbeabandonedtodefinitelypreservethetumorcapsuleandperformtotallymphadecectomy(Orchardetal.1993)2023/4/2818

SecondaryTumorsNoexperiencewithadjuvantpreandpostoperativeradiationexistsGenerallyaremultipleRadicalsurgicalresectionisoftenimpos

sibleOthertreatmentmodalitieshavetobeconsidered2023/4/2819AlternativeofCurrentTherapySurgeryRadiopharmaceutical

sCombinedChemotherapyArterialEmbolization2023/4/28202023/4/2821131I-MIBGisthetreatmentofchoiceforallunresec

table,MIBGpositivetumors58casesofmalignantPCCtreatedby131I-MIBG—therapeuticresultsandadverseevents(ZHURuisenetal.1999)2023/4/2

822Patientswereclassifiedinto3groupsaccordingtotheirtumorsize<8cm3(11cases),8～20cm3(21cases),>20cm3(26cases)Ingroup1,themeanabsorpti

ondosepergramoftumorwasabove1000cGy.Aftertreatment,tumorsdisappearedorshrinkedinallpatients2023/4/2823Ingroup2,theabsorptiondosewassimi

lartothatofgroup1,butthemeanabsorptiondosepergramwas717.6cGy,andtumormassregressionwas36%;76%reducedur

inarycatecholamineIngroup3,theabsorptiondosepergramtumortissuewas277cGy,and30%tumorenlargement,20%died;theremaining

50%symptomaticimprovementwithoutanychangeintumorsize2023/4/2824131I-MIBGisofcertaintherapeuticeffectivenes

sofsymptomaticimprovementCompletetumormassdisappearancehasonlybeenfoundinsmalltumorsTreatmentwith131I-MI

BGshouldbeinstitutedimmediatelyaftersurgicalresectiontoeradicatetheresidualtumorcellsandtopreventrecurrencesBonemarrowsuppressio

nistemporaryandnotdosagerelated2023/4/2825In1997,Lohetal.publishedareviewoftheworldwideexperienceinvolving116patientstreatedwi

th131I-MIBGformalignantpheochromocytoma.Overall,therewasasymptomaticresponsein76%,ahormonalresponsei

n45%,andtumorregressionin30%.Theactivityof131I-MIBGpersingledosewas96–300mCi,andthemeancumulativeactivitywas490±350mCi.OnlyfiveCRst

o131I-MIBGwerereported.2023/4/2826LimitationsNotallpatientswithmultiplemetastasesofmalignantpheochromoc

ytomashavesufficientuptakeofMIBGtoallowMIBGtherapyMIBGnegativelesionscoexistwithMIBGpostivelesions,requiringcombinedtreatment2023/4/2827Asa

singleagent,131I-MIBGhaslimitedefficacyintreatingmalignantpheochromocytoma.Itsuseincombinationwithothercytotoxicagents,asi

scurrentlybeingstudiedinpatientswithneuroblastoma,mayresultinadditionalbenefit(Sissonetal.1999)2023/4/2828Alternative

ofCurrentTherapySurgeryRadiopharmaceuticalsCombinedChemotherapyArterialEmbolization2023/4/2829Onlysparsedata

onchemotherapeuticregimensareavailable,mostoftheminreportsoffewcasesThemostwell-establishedregimenisCVD(Averbuchetal.1988)CTX75

0mg/m2d1,VCR1.4mg/m2d1,Dacarbazine600mg/m2d1,2Cycle21days2023/4/2830TheCVDregimenwasbasedonthetreatmentforadvancedneu

roblastoma.Thisregimenhasbeenreportedtoproducegoodresponsesinmalignantpheochromocytoma,butthemediandurationofremissionis21monthsCompletelong-ter

mdiseaseremissionswithchemotherapyhavenotbeenreported.2023/4/2831AlternativeofCurrentTherapySurgeryRadiopharma

ceuticalsCombinedChemotherapyTranscatheterArterialEmbolization2023/4/2832TAEhasbeensuccessfullyperformedinthetreatmentofmalignantPCCwithl

ivermetastasesThetherapeuticeffectsofTAEhavebeendemonstratedtobeenhancedbythecombinationtherapywithanticancerchemotherapy2023/4/

2833MitomycinChasbeensuccessfullyusedinTAEforlivermetastasisinseveralcasesofmalignantPCC.2023/4/28342023/4/2835Malignantpheochromocytoma:past,pr

esentandfuturePastPresentFutureAdrenergicblockers,α-methyl-paratyrosine&useofotherdrugsforsymptomaticreliefSurgical

debulking;131I-MIBGradiotherapy;Chemotherapy;ChemoembolizationMoleculartargeting,cancervaccines,genetherapy