葡萄膜黑色素瘤2021v2（英文）-NCCN肿瘤临床实践指南

NCCNClinicalPracticeGuidelinesinOncologyNCCNGuidelines®)Melanoma:UvealersionJuneVersion2.2021,06/25/21©2021NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.PrintedbyMinTangon9/2/202110:13:46AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2021NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.dex*SusanM.Swetter,MD/ChairϖStanfordCancerInstitute*JohnA.Thompson,MD‡†/Vice-ChairFredHutchinsonCancerResearchCenter/eCancerCareAllianceMarkR.Albertini,MD†UniversityofWisconsinCarboneCancerCenterChristopherA.Barker,MD§MemorialSloanKetteringCancerCenterJoelBaumgartner,MD¶UCSanDiegoMooresCancerCenterGenevieveBoland,MD,PhD¶MassachusettsGeneralHospitalCancerCenterBartoszChmielowski,MD,PhD‡†mprehensiveCancerCenterDominickDiMaio,MD≠Fred&PamelaBuffettCancerCenterAlisonDurham,MDϖUniversityofMichiganRogelCancerCenterRyanC.Fields,MD¶SitemanCancerCenteratBarnes-JewishHospitalandWashingtonUniversitySchoolofMedicineMartinD.Fleming,MD¶TheUniversityofTennesseealthScienceCenterAnjelaGalan,MD≠YaleCancerCenter/SmilowCancerHospitalNicoleMcMillian,MSMaiNguyenPhDnesPanelDisclosuresrianGastmanMDCaseComprehensiveCancerCenter/UniversityHospitalsSeidmanCancerlandClinicTaussigancerInstituteKennethGrossmann,MD,PhD†HuntsmanCancerInstituteattheUniversityofUtahSamanthaGuild¥AIMatMelanomaAshleyHolder,MD¶O'NealComprehensiveCancerCenteratUABDouglasJohnson,MD,MSCI†Vanderbilt-IngramCancerCenterRichardW.Joseph,MD‡†MayoClinicCancerCenterGiorgosKarakousis,MD¶AbramsonCancerCenterattheUniversityofPennsylvaniaKariKendra,MD,PhD†TheOhioStateUniversityComprehensiveCancerCenter-JamesCancerHospitalandSoloveResearchInstituteJulieR.Lange,MD,ScM¶TheSidneyKimmelComprehensiveCancerCenteratJohnsHopkinsRyanLanning,MD,PhD§UniversityofColoradoCancerCenterKimMargolin,MD†CityofHopeNationalMedicalCenterMiguelMaterin,MD۞DukeCancerInstituteAnthonyJ.Olszanski,MD,RPh†FoxChaseCancerCenterPatrickA.Ott,MD,PhD†‡ÞDana-Farber/BrighamandWomen'sCancerCenter*P.KumarRao,MD۞SitemanCancerCenteratBarnes-JewishHospitalandWashingtonUniversitySchoolofMedicineRameshRengan,MD,PhD§FredHutchinsonCancerResearchCenter/eCancerCareAllianceMerrickI.Ross,MD¶TheUniversityofTexasonCancerCenterAprilK.Salama,MD†DukeCancerInstituteohitSharmaMDhwesternSimmonsComprehensiveCancerCenterJosephSkitzki,MD¶RoswellParkCancerInstituteJeffreySosman,MD‡RobertH.LurieComprehensiveCancerCenterofNorthwesternUniversityUCSFHelenDillerFamilyComprehensiveCancerCenterEvanWuthrick,MD§MoffittCancerCenterϖDermatology‡Hematology/HematologyoncologyÞInternalmedicine†Medicaloncology۞Ophthalmology≠Pathology¥Patientadvocacy§Radiotherapy/Radiationoncology¶Surgery/Surgicaloncology*DiscussionSectionWritingCommitteeVersion2.2021,06/25/21©2021NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.PrintedbyMinTangon9/2/202110:13:46AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2021NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.dexUVEALMELANOMASUBCOMMITTEE*P.KumarRao,MD/Lead۞SitemanCancerCenteratBarnes-JewishHospitalandWashingtonUniversitySchoolofChristopherA.Barker,MD§MemorialSloanKetteringCancerCenterthaGuildAIMatMelanomaRichardJoseph,MD‡†MayoClinicCancerCenterMiguelMaterin,MD۞DukeCancerInstituteRameshRengan,MD,PhD§FredHutchinsonCancerResearchCenter/SeattleCancerCareAllianceJeffreySosman,MD‡RobertH.LurieComprehensiveCancerCenterofNorthwesternUniversity*SusanSwetter,MDϖStanfordCancerInstitute*JohnA.Thompson,MD‡†FredHutchinsonCancerResearchCenter/SeattleCancerCareAllianceEvanWuthrick,MD§MoffittCancerCenteresPanelDisclosuresϖDermatology‡Hematology/Hematologyoncology†Medicaloncology۞Ophthalmology¥Patientadvocacy§Radiotherapy/Radiationoncology*DiscussionSectionWritingCommitteeVersion2.2021,06/25/21©2021NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.PrintedbyMinTangon9/2/202110:13:46AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2021NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.dexlievesthatthebestlievesthatthebestmanagementforanypatientwithcancerisinaclinicaltrial.Participationinclinicaltrialsisespeciallyencouraged.FindanNCCNMemberInstitution:/home/member-institutions.ofEvidenceanddationsotherwisedNCategoriesofEvidenceandConsensus.NCCNCategoriesofPreference:Allrecommendationsareconsideredappropriate.SeeNCCNCategoriesofPreference.NCCNUvealMelanomaSubcommitteeMembersSummaryoftheGuidelinesUpdatesClinicalPresentation,WorkupandDiagnosis,ClinicalStaging(UM-1)WorkupandStaging,TumorSize,PrimaryTreatment(UM-2)AdditionalPrimaryTreatment(UM-3)SystemicImagingBasedonRiskStratification(UM-4)TreatmentforRecurrence(UM-5)TreatmentofMetastaticDisease(UM-6)RiskFactorsforDevelopmentofUvealMelanoma(UM-A)PrinciplesofRadiationTherapy(UM-B)SystemicTherapyforDistantMetastaticDisease(UM-C)TheNCCNGuidelinesareastatementofevidenceandconsensusoftheauthorsregardingtheirviewsofcurrentlyacceptedapproachestotreatmentAnyclinicianseekingtoapplyorconsulttheNCCNGuidelinesisexpectedtouseindependentmedicaljudgmentinthecontextofindividualstancestodetermineanypatientscareortreatmentTheNationalComprehensiveCancerNetworkNCCNmakesnorepresentationsorwarrantiesofanykindregardingtheircontentuseorapplicationanddisclaimsanyresponsibilityfortheirapplicationoruseinanywayTheNCCNbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.©2021.Version2.2021,06/25/21©2021NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.UPDATESVersion2.2021,06/25/21©2021NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.PrintedbyMinTangon9/2/202110:13:46AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2021NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.dexrsionoftheNCCNGuidelinesforMelanomaUvealfromVersioninclude•TheDiscussionhasbeenupdatedtoreflectthechangesinthealgorithm.rsionoftheNCCNGuidelinesforMelanomaUvealfromVersioninclude•TheGuidelinenamechangedfromUvealMelanomatoMelanoma:Uveal.•WorkupandDiagnosis,Firstbullet:pFirstarrowsub-bulletrevised:"H&P,includingpersonal/familyhistoryofpriororcurrentcancers(outsidetheeye)"andfootnotebadded.pThirdarrowsub-bulletrevised:Ocularultrasoundofeyeandorbit.•Footnotedrevised:"...Biopsyoftheprimarytumordoesnotimpactoutcome,butmayprovideprognosticinformationthatcanhelpinformfrequencyoffollow-upandmaybeneededforeligibilityforclinicaltrials.Ifbiopsyisperformed,molecular/chromosomaltestingforprognostication(chromosomeanalysisorgene irling[GEP])ispreferredovercytologyalone..."largestdiameterwasrevisedasfollowsmm...•Footnoteuisnew:PathologicevaluationshouldfollowtheuvealmelanomasynopticreportrecommendationsbytheCollegeofAmericanPathologists.Availableat:/protocols/cp-uveal-melanoma-17protocol-4000.pdf•Footnoteprevised:"...Thelargestcommerciallyavailablebrachytherapyplaqueis2223mmindiameter;thus,plaquebrachytherapyisrecommendedonlyfortumorswithlargestbasaldiameter≤18≤19mm.Footnotevregarding"ExtraocularextensionatthetimeofenucleationisnewThisisarelativelyrareoccurrence;dataarelimitedfortheserecommendations.•Firstcolumnrecommendationrevised:Systemicimaging±bloodtestsbasedonriskstratificationbygenetictesting±tumorsizeandhistology(atpresentation).•RiskofDistantMetastasis:UnderHighrisk,thefollowingwereremoved:pExtraocularextensionpCiliarybodyinvolvement•Footnoteddrevised:"...Additionalimagingmodalitiesmayincludechest/abdominal/pelvicCTwithcontrast,ordualenergysubtractionchestx-ray.However,screeningshould..."•Footnoteeeisnew:8qgain,especiallywhennumerouscopiesarefoundportendsgreaterriskformetastasis.•TreatmentofMetastaticDisease:pAfter"Noevidenceofdisease"revised:Clinicaltrial,ifavailable(preferred).pAfter"Residualorprogressivedisease"thearrowwasredrawnforclarity.UPDATESVersion2.2021,06/25/21©2021NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.PrintedbyMinTangon9/2/202110:13:46AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2021NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.dexrsionoftheNCCNGuidelinesforMelanomaUvealfromVersionincludeUM-ARiskFactorsforDevelopmentofUvealMelanoma•Firstbullet:pThirdarrowsub-bulletrevised:Familialuvealmelanoma(eg,germlinemutationsinBAP1mutation,PALB-2,MBD4,orNF-1(neurofibromatosis)[NF-1],dysplasticnevussyndrome[BK-mole])pThreenewarrowsub-bulletsadded:◊Highernumbersofatypicalcutaneousnevi,commoncutaneousnevi,and/orcutaneousfreckles◊Lightskincolor,propensitytosunburn,and/orlighteye(iris)color◊Strongpersonalorfamilyhistoryofcancer•Footnotebisnew:pEvaluateforevidenceofhereditarysyndromeandreferforgeneticcounselingandtestingifindicated:◊Earlyageofdiagnosis(<30yearsofage)◊Historyofotherprimarycancersinthepatient◊Familyorpersonalhistoryofothercancersknowntobeassociatedwithahereditarysyndrome:–BAP1:RCC,mesothelioma,cutaneousmelanoma,cholangiocarcinoma,meningioma–BRCA,PALB2:breast,ovarian,orpancreaticcancers•Newreferenceswereadded.PrinciplesofRadiationTherapy•TreatmentInformationpSecondarrowsub-bulletrevised:Plaquebrachytherapyisappropriateforpatientswithtumors≤18≤19mminlargestbasediameter,≤10mminthicknesspFiftharrowsub-bulletrevised:"...MRIorCTmaybeusedforpreoperativeplanning."pSixtharrowsub-bulletrevised:Roundorcustomplaquesaremostcommonlyused,.althoughnon-roundplaques(eg,notched)canbeconsideredfortumorsinspecificlocations(eg,peripapillary).Customplaques,suchasnotchedplaques,arecommonlyusedfortumorsinspecificlocations(peripapillary).•TreatmentDosingInformation,firstarrowsub-bulletrevised:"...Thelargestcommerciallyavailablebrachytherapyplaqueis2223mmindiameter;thus,plaquebrachytherapyisrecommendedonlyfortumorswithlargestbasaldiameter≤18≤19mm.2of3•Radioembolization,firstbulletrevised:Selectiveinternalradiationtherapyforpatientswithlivermetastasesusingyttrium-90hasbeenreportedinretrospectivestudiesandinoneprospectivestudy.3of3•Referencesupdated.UM-CSystemicTherapyforDistantMetastaticDisease•Preferredregimensrevised:pClinicaltrialWhenavailableandclinicallyappropriate,enrollmentinaclinicaltrialisrecommended.•Footnotearevised:Whenavailableandclinicallyappropriate,enrollmentinaclinicaltrialisrecommended.Theliteratureisnotdirectiveregardingthespecificsystemicagent(s)offeringsuperioroutcomes,butdoesprovideevidencethatuvealmelanomaissensitivetosomeofthesamesystemictherapiesusedtotreatcutaneousmelanoma.Althoughtherearenosystemictherapiesthathavereliablyimprovedtheoverallsurvivalinpatientswithmetastaticuvealmelanoma,individualpatientsmayderivesubstantialbenefitonoccasion.GiventhelackofpositivephaseIIIstudies,clinicaltrialsarepreferred.•Newreferenceswereaddedfornivolumab/ipilimumab.rgrowtheforyearsgannuallyhereafterfeaturesofmalignancyf•Every2–4monthsgasclinicallyindicated•Thenclosefollow-upCLINICALSTAGINGDiagnosisuncertainand/or<3riskfactorsObserveandre-evaluateforgrowthorSeeWorkuprgrowtheforyearsgannuallyhereafterfeaturesofmalignancyf•Every2–4monthsgasclinicallyindicated•Thenclosefollow-upCLINICALSTAGINGDiagnosisuncertainand/or<3riskfactorsObserveandre-evaluateforgrowthorSeeWorkupandStagingforuvealmelanoma(UM-2)currentcancersoutsidetheeyeborprognosticanalysisforriskstratificationdroidadexCLINICALPRESENTATIONffciliarypinclude:◊Visionloss◊Visionchanges(eg,blurredvision,photopsia,floaters,metamorphopsia)pMaybeasymptomaticpAssessmentofriskalmelanomabfactorsalmelanomabWORKUPANDDIAGNOSISWORKUPANDDIAGNOSISpColorfundusphotographypOcularultrasoundpComprehensiveeyeexam:Examinethefrontandbackofeye(biomicroscopy)◊Dilatedfundusexam(indirectophthalmoscopy)◊Measurevisualacuity◊Measureanddocumentlocationandthesizeofthetumor(diameter,thickness),distancefromdiscandfovea,andciliarybodyinvolvement◊Assessanddocumentifpresent:–Subretinalfluid–Orangepigment•Additionaltestingoptionsinclude:pAutofluorescenceoftheocularfunduspOpticalcoherencetomographypRetinalfluoresceinangiographyoftheocularfunduspTransilluminationConsiderbiopsyifneededtoconfirmConsiderbiopsyifneededtoconfirmdiagnosiscaThisguidelinedoesnotincludethemanagementofirismelanoma.bSeeRiskFactorsforDevelopmentofUvealMelanoma(UM-A).cBiopsyisusuallynotnecessaryforinitialdiagnosisofuvealmelanomaandselectionoffirst-linetreatment,butmaybeusefulincasesofuncertaintyregardingdiagnosis,suchasforamelanotictumors,orretinaldetachment.dBiopsyoftheprimarytumormayprovideprognosticinformationthatcanhelpinformfrequencyoffollow-upandmaybeneededforeligibilityforclinicaltrials.Ifbiopsyisperformed,molecular/chromosomaltestingforprognosticationispreferredovercytologyalone.Therisks/benefitsofbiopsyforprognosticanalysisshouldbecarefullyconsideredanddiscussed.eRiskfactorsforgrowthofsmallmelanocytictumors:presenceofsymptoms,tumorthickness>2mm,tumordiameter>5mm,presenceofsubretinalfluidandorangepigment,tumormarginwithin3mmofopticdisk,ultrasoundhollowness,absenceofhalo.fTherecommendationto"observeandre-evaluate"consistsoftestslistedunder"WorkupandDiagnosis"thatwouldhelptoclarifyifthereisprogressionanddeterminethenaturalhistoryoftheindeterminatelesion.gFrequencyofevaluationshoulddependonindexofsuspicion,patientage,andmedicalfrailty.Note:Allrecommendationsarecategory2Aunlessotherwiseindicated.ClinicalTrials:NCCNbelievesthatthebestmanagementofanypatientwithcancerisinaclinicaltrial.Participationinclinicaltrialsisespeciallyencouraged.UM-1Version2.2021,06/25/21©2021NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.orprognosticanalysisdrticlebeamradiationosleationtutheroptionsinhighlyselectentsrAssessanddocumentifpresent:ConsiderbiopsyofprimarytumorPrintedbyMinTangon9/2/202110:13:46AM.orprognosticanalysisdrticlebeamradiationosleationtutheroptionsinhighlyselectentsrAssessanddocumentifpresent:ConsiderbiopsyofprimarytumordexWORKUPANDSTAGINGTUMORSIZEPRIMARYTREATMENTm,n•Ocularimagingifnotpreviouslydone:pIflargetumor,closetonerveorsuspicionofextraocularwithoutIVcontrasthinvolvement,MRIofwithoutIVcontrasthpCiliarybodyinvolvementpExtraocularextension•Extraocularimaging:tdiseasehijpBaselinetdiseasehijthickness<2.5mmLargestdiameter≤19mmkLargestdiameter>19mmk[anythickness]orThickness>10mm[anydiameter]orThickness>8mmwithopticnerveinvolvement[anydiameter]onsonsrachytherapyplaqueopqs•RTleationtleationtuSeeAdditionalPrimaryTreatment(UM-3)FootnotesonUMANote:Allrecommendationsarecategory2Aunlessotherwiseindicated.ClinicalTrials:NCCNbelievesthatthebestmanagementofanypatientwithcancerisinaclinicaltrial.Participationinclinicaltrialsisespeciallyencouraged.Version2.2021,06/25/21©2021NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.PrintedbyMinTangon9/2/202110:13:46AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2021NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.dexFOOTNOTESdBiopsyoftheprimarytumormayprovideprognosticinformationthatcanhelpinformfrequencyoffollow-upandmaybeneededforeligibilityforclinicaltrials.Ifbiopsyisperformed,molecular/chromosomaltestingforprognosticationispreferredovercytologyalone.Therisks/benefitsofbiopsyforprognosticanalysisshouldbecarefullyconsideredanddiscussed.hUnlessthereisaspecificcontraindicationtotheadministrationofIVcontrast(ie,renalimpairmentorhistoryofasevereallergy),allcross-sectionalimagingstudiesshouldbeperformedwithandwithoutIVcontrast.iDespitelackoftreatmentoptionsforpatientswithdistantmetastaticdisease,NCCNfavorsstagingbeforeprimarytreatment.Forsmall,low-risktumors,imagingafterprimarytreatmentcanbeconsidered.jThemostfrequentsitesofmetastasisareliver,lungs,skin/softtissue,andbones.Atminimum,allpatientsshouldhavecontrast-enhancedMRorultrasoundoftheliver,withmodalitypreferencedeterminedbyexpertiseatthetreatinginstitution.Additionalimagingmodalitiesmayincludechest/abdominal/pelvicCTwithcontrast.However,screeningshouldlimitradiationexposurewheneverpossible.kThecutoffforlargestbasaldiameterdependsonthedimensionsofthelargestbrachytherapyplaqueavailable,somaydependonthetypeofplaqueandisotopeselectedifbrachytherapyisused.lPatientsmaybeconsideredforpalliativelocaltherapytotheprimarytumorinthesettingofmetastaticdisease.Patientswhopresentwithadvancedmetastaticdiseaseandlimitedlifeexpectancymayelecttohavenotreatmenttotheirprimarytumor.mAnessentialfeatureofhigh-qualitycareisthatclinicaldecisionsareinformedbyavarietyofcase-specificfactors(eg,patientcharacteristicsandpreferenceslikeage,statusoftheothereyeamongothers,diseasecharacteristics,medicalhistory),suchthatforsomepatientsthebestclinicalapproachmaybeotherthanoneofthelistedoptions.nForsmallciliarybodyandiristumors(lessthan3clockhours),surgicalexcisionmaybeconsidered.oSeePrinciplesofRadiationTherapy(UM-B).pTheplaqueshouldcoverthetumorwitha≥2-mmcircumferentialmargin.Theexceptionisfortumorsneartheopticnervewhereitmaybeimpossibletoachieveadequatecoverageofthemargins.Thelargestcommerciallyavailablebrachytherapyplaqueis23mmindiameter;thus,plaquebrachytherapyisrecommendedonlyfortumorswithlargestbasaldiameter≤19mm.qBrachytherapywithscleralpatchgraftforcaseswithlimitedextraocularextension.rConsiderlaserablationorenucleationforpatientswhoarenotgoodcandidatesforbrachytherapyorparticlebeamradiation.sConsideradditionaltreatmentwithresection,laserablation,transpupillarythermotherapy,orcryotherapyifconcernedthatadequateresponsewasnotachievedfrominitialradiation.tWhilethereisatrendtowardavoidingenucleation,itisrecommendedforpatientswithneovascularglaucoma,tumorreplacing>50%ofglobe,orblind,painfuleyes.Considerenucleationincasesofextensiveextraocularextension.uPathologicevaluationshouldfollowtheuvealmelanomasynopticreportrecommendationsbytheCollegeofAmericanPathologists.Availableat:/protocols/cp-uveal-melanoma-17protocol-4000.pdfNote:Allrecommendationsarecategory2Aunlessotherwiseindicated.ClinicalTrials:NCCNbelievesthatthebestmanagementofanypatientwithcancerisinaclinicaltrial.Participationinclinicaltrialsisespeciallyencouraged.UM-2AVersion2.2021,06/25/21©2021NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.rerRTtoorbitlebeamorphotonbeamoculartissueifpossiblendconsideroneormoreofthelowupllowing•Intraoperativecryotherapy•OrbitalrerRTtoorbitlebeamorphotonbeamoculartissueifpossiblendconsideroneormoreofthelowupllowing•Intraoperativecryotherapy•Orbitalexenteration•RTtoorbit(particlebeamordexADDITIONALPRIMARYTREATMENTnatthetimeoftionvallypositiveorclosesafterenucleationbutnoclinicalaoperativeorradiographicevidenceofossresidualdiseaseintheorbitVisibleextraoculartumororsuspicionofgrossdiseaseintheorbitAllothersoSeePrinciplesofRadiationTherapy(UM-B).vThisisarelativelyrareoccurrence;dataarelimitedfortheserecommendations.Note:Allrecommendationsarecategory2Aunlessotherwiseindicated.ClinicalTrials:NCCNbelievesthatthebestmanagementofanypatientwithcancerisinaclinicaltrial.Participationinclinicaltrialsisespeciallyencouraged.Version2.2021,06/25/21©2021NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.±tumorsizebefollowedwithroutineophthalmologiccare.yAdditionalriskfactorsforrecurrence:Juxtapapillarylocationandciliaryalthoughsomestudiesshowedpoorsensitivityforearlydetectionofliverientswhoelecttohavesurveillanceimagingoptionsinclude±tumorsizebefollowedwithroutineophthalmologiccare.yAdditionalriskfactorsforrecurrence:Juxtapapillarylocationandciliaryalthoughsomestudiesshowedpoorsensitivityforearlydetectionofliverientswhoelecttohavesurveillanceimagingoptionsincludecontrastenhanced(atpresentation)bbdexStandardfollow-upforaffectedeyew,x,yandSystemicimaging±bloodtestszbasedonriskgenetictestingaagenetictestingaaRISKOFDISTANTMETASTASISbb•Class1•Class1Acc•Disomy3•Gainofchromosome6p.3I」pAXmutation•T1(AJCC)(SeeST-1andST-2)•Class1•Class1Bcc.S」c日pmutation•T2andT3(AJCC)(SeeST-1andST-2)•Class2•Class2cc•Monosomy3•Gainofchromosome8qee.日Adpmutation.dRAM3expression•T4(AJCC)(SeeST-1andST-2)SYSTEMICIMAGINGBASEDONRISKSTRATIFICATION•Considersurveillanceimagingddevery12monthsevery3–6monthsfor5•Considersurveillanceimagingddevery12monthsevery3–6monthsfor5years,theneverymonthsforyears,thenasclinicallyindicated•Considersurveillanceim