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,MPHFÞeneralHospitalenterRobertBaiocchi,MD,PhD†TheOhioStateUniversityComprehensiveCancerCenter-JamesCancerHospitalandSoloveResearchInstituteEvieCarchman,MD¶UniversityofWisconsineCancerCenterScottChristensen,MD†UCDavisComprehensiveCancerCenterOxanaV.CryslerMD†UniversityofMichiganRogelCancerCenterFreedmanCassPhDchonfeldBAesPanelDisclosuresGauravGoyal,MD†‡ÞO'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†ÞSitemanCancerCenteratBarnes-JewishHospitalandWashingtonUniversitySchoolofMedicineStaceyRizza,MDFCancerCenterJulianSanchez,MD¶MoffittCancerCenterJeffTaylor¥HIV+AgingResearchProject-PalmSpringsJohnTimmerman,MD†UCLAJonssonComprehensiveCancerCenterBenjaminTomlinson,MD†‡CaseComprehensiveCancerCenter/UniversityHospitalsSeidmanCancerCenterandClevelandClinicTaussigCancerInstituteChia-ChingJ.Wang,MD†UCSFHelenDillerFamilyComprehensiveCancerCenterAnjanaV.Yeldandi,MD≠RobertH.LurieComprehensiveCancerCenterofNorthwesternUniversityξBonemarrowtransplantation‡Hematology/HematologyoncologyFInfectiousdiseasesÞInternalmedicine†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