葡萄膜黑色素瘤2021v2（英文）-NCCN腫瘤臨床實踐指南

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/Chair?StanfordCancerInstitute*JohnA.Thompson,MD??/Vice-ChairFredHutchinsonCancerResearchCenter/eCancerCareAllianceMarkR.Albertini,MD?UniversityofWisconsinCarboneCancerCenterChristopherA.Barker,MD§MemorialSloanKetteringCancerCenterJoelBaumgartner,MD?UCSanDiegoMooresCancerCenterGenevieveBoland,MD,PhD?MassachusettsGeneralHospitalCancerCenterBartoszChmielowski,MD,PhD??mprehensiveCancerCenterDominickDiMaio,MD≠Fred&PamelaBuffettCancerCenterAlisonDurham,MD?UniversityofMichiganRogelCancerCenterRyanC.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??TDana-Farber/BrighamandWomen'sCancerCenter*P.KumarRao,MD?SitemanCancerCenteratBarnes-JewishHospitalandWashingtonUniversitySchoolofMedicineRameshRengan,MD,PhD§FredHutchinsonCancerResearchCenter/eCancerCareAllianceMerrickI.Ross,MD?TheUniversityofTexasonCancerCenterAprilK.Salama,MD?DukeCancerInstituteohitSharmaMDhwesternSimmonsComprehensiveCancerCenterJosephSkitzki,MD?RoswellParkCancerInstituteJeffreySosman,MD?RobertH.LurieComprehensiveCancerCenterofNorthwesternUniversityUCSFHelenDillerFamilyComprehensiveCancerCenterEvanWuthrick,MD§MoffittCancerCenter?Dermatology?Hematology/HematologyoncologyTInternalmedicine?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,MD?StanfordCancerInstitute*JohnA.Thompson,MD??FredHutchinsonCancerResearchCenter/SeattleCancerCareAllianceEvanWuthrick,MD§MoffittCancerCenteresPanelDisclosures?Dermatology?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