卡波氏肉瘤2022.v1-NCCN（英文版）

NCCNClinicalPracticeGuidelinesinOncologyNCCNGuidelines?)KaposiSarcomarsionFebruaryVersion1.2022,2/3/22?2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.PrintedbyMinTangon3/14/20227:30:36AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright?2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.adex*ErinReid,MD/Co-chair?UCSanDiegoMooresCancerCenter*GitaSuneja,MD/Co-chair§HuntsmanCancerInstituteattheUniversityofUtahRamiAl-Rohil,MBBS≠DukeCancerInstituteRichardF.Ambinder,MD,PhD?TheSidneyKimmelComprehensiveCancerCenteratJohnsHopkinsKevinArdMD,MPHFTeneralHospitalenterRobertBaiocchi,MD,PhD?TheOhioStateUniversityComprehensiveCancerCenter-JamesCancerHospitalandSoloveResearchInstituteEvieCarchman,MD?UniversityofWisconsineCancerCenterScottChristensen,MD?UCDavisComprehensiveCancerCenterOxanaV.CryslerMD?UniversityofMichiganRogelCancerCenterFreedmanCassPhDchonfeldBAesPanelDisclosuresGauravGoyal,MD??TO'NealComprehensiveCancerCenteratUABNeelGupta,MD?StanfordCancerInstituteDavidH.Henry,MD?AbramsonCancerCenterattheUniversityofPennsylvaniaAmyJones,MD?UTSouthwesternSimmonsComprehensiveCancerCenterAnnKlopp,MD,PhD§TheUniversityofTexasAnnS.LaCasce,MD?Dana-Farber/BrighamandWomen'sCancerCenterChiLin,MD,PhD§Fred&PamelaBuffettCancerCenterManojP.Menon,MD,MPH?FredHutchinsonCancerResearchCenter/SeattleCancerCareAllianceDavidMorgan,MD?ξVanderbilt-IngramCancerCenterNityaNathwani,MD?CityofHopeNationalMedicalCenterHenryS.Park,MD,MPH§YaleCancerCenter/SmilowCancerHospitalLeeRatner,MD,PhD?TSitemanCancerCenteratBarnes-JewishHospitalandWashingtonUniversitySchoolofMedicineStaceyRizza,MDFCancerCenterJulianSanchez,MD?MoffittCancerCenterJeffTaylor￥HIV+AgingResearchProject-PalmSpringsJohnTimmerman,MD?UCLAJonssonComprehensiveCancerCenterBenjaminTomlinson,MD??CaseComprehensiveCancerCenter/UniversityHospitalsSeidmanCancerCenterandClevelandClinicTaussigCancerInstituteChia-ChingJ.Wang,MD?UCSFHelenDillerFamilyComprehensiveCancerCenterAnjanaV.Yeldandi,MD≠RobertH.LurieComprehensiveCancerCenterofNorthwesternUniversityξBonemarrowtransplantation?Hematology/HematologyoncologyFInfectiousdiseasesTInternalmedicine?Medicaloncology≠Pathology￥Patientadvocacy§Radiotherapy/Radiationoncology?Surgery/Surgicaloncology*DiscussionWritingCommitteeMemberVersion1.2022,2/3/22?2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.PrintedbyMinTangon3/14/20227:30:36AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright?2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.adexFindanNCCNMemberInstitution:/home/member-institutions.dNCCNCategoriesofPreference:Allrecommendationsareconsideredappropriate.SeeNCCNCategoriesofPreference.SummaryofGuidelinesUpdates?DiagnosisandWorkup(KS-1)?LimitedCutaneousDisease(KS-2)?AdvancedCutaneous,Oral,Visceral,orNodalDisease(KS-3)?Surveillance(KS-4)?StagingClassificationandResponseDefinitionsforKS(KS-A)?PrinciplesandGoalsofTherapy(KS-B)?PrinciplesofImmuneReconstitutionInflammatorySyndrome(IRIS)(KS-C)?LocalTherapy(KS-D)?PrinciplesofRadiationTherapy(KS-E)?SystemicTherapy(KS-F)TheNCCNGuidelinesareastatementofevidenceandconsensusoftheauthorsregardingtheirviewsofcurrentlyacceptedapproachestotreatmentAnyclinicianseekingtoapplyorconsulttheNCCNGuidelinesisexpectedtouseindependentmedicaljudgmentinthecontextofindividualstancestodetermineanypatientscareortreatmentTheNationalComprehensiveCancerNetworkNCCNmakesnorepresentationsorwarrantiesofanykindregardingtheircontentuseorapplicationanddisclaimsanyresponsibilityfortheirapplicationoruseinanywayTheNCCNbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.?2022.Version1.2022,2/3/22?2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.UPDATESVersion1.2022,2/3/22?2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.every3weeksor60mg/m2IVweeklywithdexamethasonePrintedbyMinevery3weeksor60mg/m2IVweeklywithdexamethasoneadexoftheNCCNGuidelinesforKaposiSarcomafromVersionincludeWorkup:Essential?5thbulletrevised:HIVdiagnostictesting,ifnotalreadyperformed,inpeoplewithHIV(PWH)?8thbulletrevised:Pregnancytestinginwomenpatientsofchildbearingagepotential(ifchemotherapyorradiationtherapy[RT])KS-2?Footnotefrevised:AllPWHwhohavelimitedcutaneousdiseasethatissymptomaticand/orcosmeticallyunacceptableshouldreceiveARTwithorwithoutanotherfirst-linetherapy...SeePrinciplesofImmuneReconstitutionInflammatorySyndrome(IRIS)(KS-C).[AlsoappliestoKS-3.]KS-4ance?3rdbulletrevised:Ifsignsandsymptomsconcerningforvisceralinvolvementorpriortonewtherapyifprogression/refractorydiseaseorifchangeindiseaseisnotedp3rdsub-bulletadded:PET/CT?Footnotebadded:ImagingshouldbedirectedbysymptomsorfindingsconcerningforvisceralorboneinvolvementaswellascoexistingKICS,MCD,orHHV8+lymphoma;imagingisstandardforstagingoftransplant-associatedKS.KS-ConaddedPrinciplesofImmuneReconstitutionInflammatoryromeIRISPrinciplesofRadiationTherapy?GeneralTreatmentInformationpDosingPrescriptionRegimen?5thsub-bulletadded:40Gyin20fractions?Footnote5added:StelzerKJ,GriffinTW.ArandomizedprospectivetrialofradiationtherapyforAIDS-associatedKaposi'ssarcoma.IntJRadiatOncolBiolPhys1993;27:1057-1061.KS-F(1of3)SystemicTherapyFirst-linesystemictherapyoptions?Preferredregimensp2ndsub-bulletadded:FortransplantKS,sirolimusaddedasacategory2A,preferredrecommendationKS-F(2of3)SystemicTherapyDosingFirst-LineSystemicTherapyDosingPreferredregimensp2ndbulletadded:Sirolimus(fortransplantKS)dosingadded:?sub-sub-bulletadded:Loadingdose0.15mg/kgPOfollowedby0.04–0.06mg/kg/daytomaintaintroughbloodlevelsof6–10ng/mLPaclitaxeladditionalPaclitaxeladditionaldosingaddedmaynotbeneeded;ifused,thedoseshouldbeminimizedandtailoredtopatientneeds.SubsequentSystemicTherapyOptionsforRelapsed/RefractoryTherapyDosing?OtherrecommendedregimenspBortezomib?Sub-bulletrevised:1.6mg/m2IV/SCondays1,8,and15ofeach28-daycycleKS-F(3of3)SystemicTherapyReferences?PaclitaxelpAdded:BaskanEB,etal.TreatmentofadvancedclassicKaposi’ssarcomawithweeklylow-dosepaclitaxeltherapy.IntJDermatol2006;45:1441-1443.pAdded:PatelN,etal.Successfultreatmentofpost-renaltransplantKaposi'ssarcomawithPaclitaxel.AmJTransplant2002;2:877-879.olhemoccultChestx-rayPhotographyoforalconjunctival,andcutaneousnswithreferenceunitofmeasureinthepicturefordocumentationofextentofdiseasePregnancytestinginpatientsofchildbearingpotentialChestCTwithcontrastolhemoccultChestx-rayPhotographyoforalconjunctival,andcutaneousnswithreferenceunitofmeasureinthepicturefordocumentationofextentofdiseasePregnancytestinginpatientsofchildbearingpotentialChestCTwithcontrastabdominal/pelvicCTwithcontrastorMRIwithcontrastand/orPET/CTscanbUpperendoscopyEGDcolonoscopyifgastrointestinalptomsorpositivehemoccultBronchoscopyifunexplainedpulmonarysymptomsorabnormalitiesonchestxrayorCTMRIcicechocardiogramifanthracyclineplanneddpericardialeffusionoexistingKSHVassociateddiseasesc?ReviewofallslideswithatleastoneparaffinblockrepresentativeofthetumorbyapathologistwithexpertiseinthediagnosisofKaposisarcoma(KS)pRebiopsyifnon-diagnostic?Histopathologyreviewofadequatebiopsy(ie,skinpunch,incisional,excisional)?Adequateimmunophenotypingtoestablishdiagnosis?Immunohistochemistry(IHC)panel:Kaposisarcoma-associatedherpesvirus(KSHV;humanherpesvirus8[HHV-8])LANA-1CompletebloodcountCBCdifferentialandhensivemetabolicpanelEvaluationforsuspectedopportunisticinfections(OIs)astxaminationsanddocumentationofedemachastransplantglucocorticoidsadexWORKUPDIAGNOSISKSSTAGEWORKUPDIAGNOSISESSENTIAL:cutaneousdiseaseSeeFirst-LineTherapy(KS-2)ifchemotherapyorradiationtherapyifchemotherapyorradiationtherapyRTplanned?IHC:CD31andCD34ifunclearwhetherthetumorhasavascularorigin?Encourageadditionalbiopsyofnodalorvisceralsitesifacoexistingdisorderissuspected(ie,infection,lymphoma,multicentricCastlemandisease)aAllpatientswhoareHIVseropositiveshouldhaverecentT-cellsubsets,includingquantitativeCD4+T-cellcountandHIVviralloadtoassessimmunefunctionandHIVcontrol(seeDiscussion).Involvementofaninfectiousdisease(ID)specialisttoevaluateforcoexistingOIisappropriate,especiallywithadvancedimmunosuppression.bImagingshouldbedirectedbysymptomsorfindingsconcerningforvisceralorboneinvolvementaswellascoexistingKSHV-associatedinflammatorycytokinesyndrome(KICS),multicentricCastlemandisease(MCD),orHHV8+lymphoma;imagingisstandardforstagingoftransplant-associatedKS.cUsefulinpatientswithclinicalfeatures(ie,fever,dyspnea,effusions)concerningforKICSorKSHV-associatedMCD:C-reactiveprotein,KSHVserumviralload,serumproteinelectrophoresis(SPEP),IL-6,orIL-10.dSeeStagingClassificationforKS(KS-A,1of2)andResponseDefinitionsforKS(KS-A,2of2)Note:Allrecommendationsarecategory2Aunlessotherwiseindicated.ClinicalTrials:NCCNbelievesthatthebestmanagementofanypatientwithcancerisinaclinicaltrial.Participationinclinicaltrialsisespeciallyencouraged.KS-1Version1.2022,2/3/22?2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.TopicalsiorIntralesionalPWHARTf,gchemotherapyidiseaseorResponsenRTe,jororCryotherapyClinicaltriallwithHIV.iSeeLocalTherapy(KS-D).jSeePrinciplesofRadiationTherapy(KS-E)TopicalsiorIntralesionalPWHARTf,gchemotherapyidiseaseorResponsenRTe,jororCryotherapyClinicaltriallwithHIV.iSeeLocalTherapy(KS-D).jSeePrinciplesofRadiationTherapy(KS-E).kSeeSystemicTherapy(KS-F).ialsgoviveorrelapseddiseaseevaluateforinadequateHIVcontrolARTfailureasfactortoinadequateKScontrolandaddresspossiblechangeinARTinwithanHIVspecialistSeeNCCNGuidelinesforCancerinPeoplewithHIVsponsetotherapyKSrelapsesorprogressesrepeatuseofpreviouslyeffectivetherapymaybeconsidered,particularlyifresponsewasdurable.ithHIVfAllPWHwhohavelimitedcutaneousdiseasethatissymptomaticand/orcosmeticallyunacceptableshouldreceiveARTwithorwithoutanotherfirst-linetherapy.InitiationofARTmayresultinimmunereconstitutioninflammatorysyndrome(IRIS)within3–6months;IRISischaracterizedbymarkedlesionalswelling,increasedtenderness,andperipheraledema.However,ARTshouldnotbedelayedordiscontinuedunlesslife-threateningIRISdevelops.ReconstitutionofimmunefunctionisimportantforobtainingandmaintainingcontrolorremissionofKS.SeePrinciplesofImmuneReconstitutionInflammatorySyndrome(IRIS)(KS-C).gGlucocorticoidsinanyformulationshouldbeavoidedduetotheirassociationwithKSprogression.However,incasesoflife-threateningconditions,theirusemaybeconsidered.adexKSSTAGEd,hFIRST-LINETHERAPYeRESPONSEdRELAPSED/REFRACTORYTHERAPYticrllyableanceARTf,gforPWHARTf,gforPWHctherapykProgressivectherapyksymptomaticsymptomaticllyletomaticandorllyunacceptableveStartorContinuefor(PWH)hOncologyandHIVcliniciansalongwithbothanoncologypharmacistandHIVdSeeStagingClassificationforhOncologyandHIVcliniciansalongwithbothanoncologypharmacistandHIVableshouldreviewproposedcancertherapysupportivecareableshouldreviewproposedcancertherapysupportivecarensandARTforpossibledrugtodruginteractionsDDIsandoverlappingtoxicitiespriortoinitiationCo-managementbyanoncologistandanHIVclinicianisendedforthedurationoftherapySeeNCCNGuidelinesforCancerinPeopleeSeePrinciplesandGoalsofTherapy(KS-B).Note:Allrecommendationsarecategory2Aunlessotherwiseindicated.ClinicalTrials:NCCNbelievesthatthebestmanagementofanypatientwithcancerisinaclinicaltrial.Participationinclinicaltrialsisespeciallyencouraged.KS-2Version1.2022,2/3/22?2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.PrintedbyMinTangon3/14/20227:30:36AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright?2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.adexKSSTAGEd,hdvancedcutaneous,oral,visceral,oricherapyicherapymicialliallforPWHTHERAPYeapyapykRESPONSEdanceandContinueARTfRESPONSEdanceandContinueARTf,gforPWHediseasemResponseediseasemResponseClinicalClinicaltriallorSeeRelapsed/RefractoryorprogressivediseasemDiseaseTherapym(KS-F)orprogressivediseasemforPWHeligibilityforemicrapymictherapyNoteligibleforsystemictherapy above BestsupportivecaredSeeStagingClassificationforKS(KS-A1of2)andResponseDefinitionsforKS(KS-A,2of2).eSeePrinciplesandGoalsofTherapy(KS-B).fAllPWHwhohavelimitedcutaneousdiseasethatissymptomaticand/orcosmeticallyunacceptableshouldreceiveARTwithorwithoutanotherfirst-linetherapy.InitiationofARTmayresultinimmunereconstitutioninflammatorysyndrome(IRIS)within3–6months;IRISischaracterizedbymarkedlesionalswelling,increasedtenderness,andperipheraledema.However,ARTshouldnotbedelayedordiscontinuedunlesslife-threateningIRISdevelops.ReconstitutionofimmunefunctionisimportantforobtainingandmaintainingcontrolorremissionofKS.SeePrinciplesofImmuneReconstitutionInflammatorySyndrome(IRIS)(KS-C).gGlucocorticoidsinanyformulationshouldbeavoidedduetotheirassociationwithKSprogression.However,incasesoflife-threateningconditions,theirusemaybeconsidered.hOncologyandHIVclinicians,alongwithbothanoncologypharmacistandHIVpharmacist,ifavailable,shouldreviewproposedcancertherapy,supportivecaremedications,andARTforpossibleDDIsandoverlappingtoxicitiespriortoinitiation.Co-managementbyoncologistandHIVclinicianisrecommendedforthedurationoftherapy.SeeNCCNGuidelinesforCancerinPeoplewithHIV.jSeePrinciplesofRadiationTherapy(KS-E).kSeeSystemicTherapy(KS-F).lSeeclinical.mIfprogressiveorrelapseddisease,evaluateforinadequateHIVcontrol/ARTfailureasacontributingfactortoinadequateKScontrolandaddresspossiblechangeinARTinconjunctionwithanHIVspecialist.SeeNCCNGuidelinesforCancerinPeoplewithHIV.oSystemictherapyispreferredoverradiationtherapyasfirst-linetherapyandrelapsed/refractorytherapyfordisseminateddiseasewheneversystemictherapyisfeasibleconsideringperformancestatusandcomorbidities.Note:Allrecommendationsarecategory2Aunlessotherwiseindicated.ClinicalTrials:NCCNbelievesthatthebestmanagementofanypatientwithcancerisinaclinicaltrial.Participationinclinicaltrialsisespeciallyencouraged.KS-3Version1.2022,2/3/22?2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.PrintedbyMinTangon3/14/20227:30:36AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright?2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.adexSURVEILLANCE?ForpatientsnotrequiringactivetherapyandwithnosignsofprogressionpFollow-upperiodicallybasedonresponsetotherapyand,ifapplicable,degreeofHIVviremiaandimmunereconstitution?Historyandphysicalexamination–includinghistoryofadditionalimmunosuppressionsuchastransplant/glucocorticoids–includingcompleteskinandoralexaminations,anddocumentationofedema?CBC,differential,comprehensivemetabolicpanel?PWH–T-cellsubsets(CD4+T-cellcount)andHIVviralload–AssessARTcompliance?Photographyoforal,conjunctival,andcutaneouslesions(withreferenceunitofmeasureinthepicture)fordocumentationofextentofdiseaseifchangeindiseaseisnoted?Ifsignsandsymptomsconcerningforvisceralinvolvementorpriortonewtherapyifprogression/refractorydiseaseorifchangeindiseaseisnotedpendingonclinicalconcernsb?AsKSHVisnoteradicatedwithtreatmentofKS,theriskforfutureKSpersistsevenaftercompleteremission.?ForPWH,optimizationandmonitoringofHIVcontrolandimmunefunctionisimportanttominimizethisrisk.Thisriskdependsonimmunefunctionandgenerallydecreaseswithimmunereconstitution.However,KScanpersist,relapse,orpresenteveninthesettingofnormalvaluesofT-cellsubsets.Lessfrequent(every6–12mo)oncologymonitoringmaybeappropriateforselectedpatientswithundetectableHIVviralloads,normalT-cellsubsets,andstableKSfor2ormoreyearsaslongasthepatienthasregularfollow-upwithanHIVprovider.bImagingshouldbedirectedbysymptomsorfindingsconcerningforvisceralorboneinvolvementaswellascoexistingKICS,MCD,orHHV8+lymphoma;imagingisstandardforstagingoftransplant-associatedKS.Note:Allrecommendationsarecategory2Aunlessotherwiseindicated.ClinicalTrials:NCCNbelievesthatthebestmanagementofanypatientwithcancerisinaclinicaltrial.Participationinclinicaltrialsisespeciallyencouraged.KS-4Version1.2022,2/3/22?2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.PrintedbyMinTangon3/14/20227:30:36AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright?2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.adexSTAGINGCLASSIFICATIONFORKSaGoodrisk(allofthefollowing)Poorrisk(anyofthefollowing)TConfinedtoskinand/orlymphnodesandorminimaloraldisease(non-nodularonfinedtopalateT1:Tumor-associatededemaorulcerationExtensiveoralKSGastrointestinalKSnsotherthanlymphnodesmIntLntLSystemicdisease,SS0:NohistoryofopportunisticinfectionorthrushNo“B”symptoms2KarnofskyPerformanceStatus≥70S1:Historyofopportunisticinfectionand/orthrush“B”symptomspresentKarnofskyPerformanceStatus<70OtherHIV-relatedillness(eg,neurologicdisease,lymphoma)1IstagehaslessprognosticvaluethanTorSstagesinpatientsonARTtherapy.2“B”symptomsareunexplainedfever,nightsweats,>10%involuntaryweightloss,ordiarrheapersisting>2weeks.aAdaptedfromKrownSEMetrokaCWernzJCKaposissarcomaintheacquiredimmunedeficiencysyndrome:aproposalforuniformevaluation,response,andstagingcriteriaAIDSClinicalTrialsGroupOncologyCommitteeJClinOncol1989;7:1201-1207.Note:Allrecommendationsarecategory2Aunlessotherwiseindicated.ClinicalTrials:NCCNbelievesthatthebestmanagementofanypatientwithcancerisinaclinicaltrial.Participationinclinicaltrialsisespeciallyencouraged.Version1.2022,2/3/22?2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.KSAOF2PrintedbyMinTangon3/14/20227:30:36AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright?2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.adexRESPONSEDEFINITIONSFORKSasponseTheabsenceofanydetectableresidualdisease,includingtumor-associated(local)edema,persistingforatleast4weeks.Patientsknowntohavehadvisceraldiseaseshouldhaverestagingwithappropriateendoscopicorradiographicproceduresrelevanttositesinvolvedatbaseline.eNonewmucocutaneouslesionsvisceralsitesofinvolvementortheappearanceorworseningofatededemaoreffusionsANDpA50%orgreaterdecreaseinthenumberofallpreviousexistinglesionslastingforatleast4weeks;ORpCompleteflatteningofatleast50%ofallpreviouslyraisedlesions(ie,50%ofallpreviouslynodularorplaque-likelesionsbecomemacules);ORpA50%decreaseinthesumoftheproductsofthelargestperpendiculardiametersofatleast5measurablelesions.enthereisresidualtumorassociatededemaoreffusionbutdiseaseotherwisemeetscriteriaforcompleteresponse,responseshouldbeclassifiedas"partial."blediseaseSDAnyresponsethatdoesnotmeetthecriteriaforprogressivedieaseorPR.eAnincreaseof>25%inthesizeofpre-existinglesionsand/ortheappearanceofnewlesionsorsitesofdiseaseandorachangeinthecharacteroftheskinororallesionsfrommaculartoplaque-likeornodularofIfneworincreasingtumorassociatededemaoreffusiondevelop,diseaseisconsideredtobeprogressive.aAdaptedfromKrownSEMetrokaCWernzJCKaposissarcomaintheacquiredimmunedeficiencysyndrome:aproposalforuniformevaluation,response,andstagingcriteraAIDSClinicalTrialsGroupOncologyCommitteeJClinOncol1989;7:1201-1207.Note:Allrecommendationsarecategory2Aunlessotherwiseindicated.ClinicalTrials:NCCNbelievesthatthebestmanagementofanypatientwithcancerisinaclinicaltrial.Participationinclinicaltrialsisespeciallyencouraged.Version1.2022,2/3/22?2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.KSA2OF2PrintedbyMinTangon3/14/20227:30:36AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright?2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.adexPRINCIPLESANDGOALSOFTHERAPYPrinciplesofTherapy:?IndividualKSlesionsmaybedistinctclonesthatariseduetothecommonriskfactorsofimmunosuppressionandpersistentKSHVinfectionasopposedtometastases.Treatmentofexistingdiseasethereforemaynotpreventoccurrenceoffuturelesions.?Optimizationofimmunefunctionandavoidanceofadditionalimmunosuppressionarecriticaltopreventionofadditio