肝胆癌2022.v1（英文）-NCCN肿瘤临床实践指南

NCCNClinicalPracticeGuidelinesinOncologyNCCNGuidelines®)HepatobiliaryCancersersionMarchNCCNGuidelinesforPatients®availableat/patientsVersion1.2022,03/29/22©2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.CenterMassachusettsGeneralPrintedbyMinTangon4/1/202211:01:21AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2022NationalComprehensiveCancerNetwork,Inc.,AllRightsCenterMassachusettsGeneraldex*AlB.Benson,III,MD/Chair†RobertH.LurieComprehensiveCancerCenterofNorthwesternUniversity*MichaelI.D’Angelica,MD/Vice-Chair¶MemorialSloanKetteringCancerCenterDanielE.Abbott,MD¶UniversityofWisconsinCarboneCancerCenterDanielA.Anaya,MD¶MoffittCancerCenterRobertAnders,MD,PhD¤≠TheSidneyKimmelComprehensiveCancerCenteratJohnsHopkinsChandrakanthAre,MD,MBA¶Fred&PamelaBuffettCancerCenterieCholangiocarcinomaFoundationMiteshBorad,MD†MayoClinicCancerCenterDanielBrown,MD†фVanderbilt-IngramCancerCenterAdamBurgoyne,MD,PhD†USSanDiegoMooresCancerCenterenChahalMDrehensiveCancerCenteriversityHospitalsSeidmanCancerCenternstituteDanielT.Chang,MD§StanfordCancerInstituteJordanCloyd,MD¶TheOhioStateUniversityComprehensiveCancerCenter-JamesCancerHospitalandSoloveResearchInstituteerPhDcMillianMSchonfeldBAAnneM.Covey,MDфMemorialSloanKetteringCancerCenterSepidehGholami,MD¶UCDavisComprehensiveCancerCenterEvanS.Glazer,MD,PhD¶St.JudeChildren'sResearchHospital/TheUniversityofTennesseeHealthScienceCentermandWomensamandWomenserCenterWilliamG.Hawkins,MD¶SitemanCancerCenteratBarnes-JewishHospitalandWashingtonUniversitySchoolofMedicineRenukaIyer,MDÞ†RoswellParkComprehensiveCancerCenterRojymonJacob,MD§O'NealComprehensiveCancerCenteratUABLawrenceJennings,MD,PhD≠RobertH.LurieComprehensiveCancerCenterofNorthwesternUniversityR.KateKelley,MD†‡UCSFHelenDillerFamilyComprehensiveCancerCenterRobinKim,MDξ¶HuntsmanCancerInstituteattheUniversityofUtahesPanelDisclosuresMatthewLevine,MD,PhDξAbramsonCancerCenterattheUniversityofPennsylvaniaManishaPalta,MD§DukeCancerInstituteJamesO.Park,MD¶FredHutchinsonCancerResearchCenter/SeattleCancerCareAllianceStevenRaman,MDфUCLAJonssonComprehensiveCancerCenterSanjayReddy,MD,FACS¶FoxChaseCancerCenterVaibhavSahai,MD,MS†UniversityofMichiganRogelCancerCenterTraceySchefter,MD§UniversityofColoradoCancerCenterGagandeepSingh,MD¶CityofHopeNationalMedicalCenterStaceyStein,MD†YaleCancerCenter/SmilowCancerHospitalJean-NicolasVauthey,MD¶TheUniversityofTexasMDAndersonCancerCenterAlanP.Venook,MD†‡ÞUCSFHelenDillerFamilyComprehensiveCancerCenterAdamYopp,MD¶UTSouthwesternSimmonsComprehensiveCancerCenter¤Gastroenterology‡Hematology/HematologyoncologyÞInternalmedicineфInterventionalradiology†Medicaloncology¥Patientadvocacy≠Pathology§Radiotherapy/Radiationoncology¶Surgery/SurgicaloncologyξTransplantation*DiscussionsectionwritingcommitteeVersion1.2022,03/29/22©2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.•HCCScreening(HCC-1)•DiagnosisofHCC(HCC-2)•ClinicalPresentationandWorkup:•HCCScreening(HCC-1)•DiagnosisofHCC(HCC-2)•ClinicalPresentationandWorkup:HCCConfirmed(HCC-3)•PotentiallyResectableorTransplantable,OperablebyPerformanceStatusorComorbidity(HCC-4)•UnresectableHepatocellularCarcinoma(HCC-5)•Liver-ConfinedDisease,InoperablebyPerformanceStatus,Comorbidity,orWithMinimalorUncertainExtrahepaticDisease(HCC-6)•PrinciplesofImaging(HCC-A)•PrinciplesofCoreNeedleBiopsy(HCC-B)•Child-PughScore(HCC-C)•PrinciplesofSurgery(HCC-D)•PrinciplesofLocoregionalTherapy(HCC-E)•PrinciplesofRadiation(HCC-F)•PrinciplesofSystemicTherapy(HCC-G)BiliaryTractCancers:GallbladderCancer•IncidentalFindingatSurgery(GALL-1)•IncidentalFindingonPathologicReview(GALL-2)•MassonImaging(GALL-3)•JaundiceandMetastaticDisease(GALL-4)•Post-Resection(GALL-5)•PrinciplesofSurgeryandPathology(GALL-A)BiliaryTractCancers:IntrahepaticCholangiocarcinoma•Presentation,Workup,PrimaryTreatment(INTRA-1)•AdjuvantTreatment,Surveillance(INTRA-2)•PrinciplesofSurgery(INTRA-A)ractCancersExtrahepaticCholangiocarcinoma•Presentation,Workup,PrimaryTreatment(EXTRA-1)•AdjuvantTreatment,Surveillance(EXTRA-2)•PrinciplesofSurgery(EXTRA-A)BiliaryTractCancers•PrinciplesofImaging(BIL-A)•PrinciplesofRadiationTherapy(BIL-B)•PrinciplesofSystemicTherapy(BIL-C)HepatobiliaryCancers•AJCCStaging(ST-1)•BCLCStaging(ST-6)dexlievesthatthebestmanagementforanypatientwithcancerisinaclinicaltrial.Participationinclinicaltrialsisespeciallyencouraged.FindanNCCNMemberInstitution:/home/member-institutions.ofEvidenceanddationsotherwisedNCategoriesofEvidenceandConsensus.NCCNCategoriesofPreference:Allrecommendationsareconsideredappropriate.SeeNCCNCategoriesofPreference.TheNCCNGuidelinesareastatementofevidenceandconsensusoftheauthorsregardingtheirviewsofcurrentlyacceptedapproachestotreatmentAnyclinicianseekingtoapplyorconsulttheNCCNGuidelinesisexpectedtouseindependentmedicaljudgmentinthecontextofindividualstancestodetermineanypatientscareortreatmentTheNationalComprehensiveCancerNetworkNCCNmakesnorepresentationsorwarrantiesofanykindregardingtheircontentuseorapplicationanddisclaimsanyresponsibilityfortheirapplicationoruseinanywayTheNCCNbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.©2022.Version1.2022,03/29/22©2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.UPDATESVersion1.2022,03/29/22©2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.PrintedbyMinTangon4/1/202211:01:21AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.dexersionoftheNCCNGuidelinesforHepatobiliaryCancersfromVersioninclude•HepatocellularCarcinomaScreeningpColumn2,footnoteamoved:FromAlphafetoprotein(AFP)toUltrasound(US).pFootnoteeadded:NiaziSK,etal.JNatlComprCancNetw2021;19:829-838.•DiagnosisofHCCpFootnoteprevised:Theoptimaldiagnosticmethodiscoreneedlebiopsy.SeePrinciplesofCoreNeedleBiopsy(HCC-B).(AlsoforHCC-6)HCC-3•Column3,pathway4revised:Metastaticdiseaseorextensivelivertumorburden.•SurgicalAssessmentpColumn3,bullet2revised,ConsiderbBridgetherapyasindicated.ncepBullet1revised,"...every3–6mofor2y,thenevery6–12mo".(AlsoforHCC-5)pBullet2revised:"...every3–6mofor2y,thenevery6–12mo".(AlsoforHCC-5)pFootnotexrevised:InhighlyselectedpatientswithChild-PughClassBliverfunctionpatientswithlimitedresection.•Footnotezadded:ConsiderbiopsyifimagingisnotconsistentortoconfirmimagingdiagnosisifitdoesnotmeetAASLDorLIRADS-5criteria.SeePrinciplesofImaging(HCC-A).Theoptimaldiagnosticmethodiscoreneedlebiopsy.SeePrinciplesofCoreNeedleBiopsy(HCC-B).(AlsoforHCC-5)•Footnoteggadded:SurveillanceimagingandAFPshouldcontinueforatleast5yearsandthereafterscreeningdependentonHCCriskfactors.SeePrinciplesofImaging(SeeHCC-A).(AlsoforHCC-5)ablepColumn2revised:Evaluatewhetherpatientisacandidatefortransplant[SeeUNOS/extendedcriteriaunderSurgicalAssessment.pTreatment,transplantcandidate,bullet2revised:ConsiderBridgetherapyasindicated.•Footnotekkadded:SeeNCCNGuidelinesforPalliativeCare.(AlsoforHCC-6)HCC-A1of3•Screeningandsurveillance,bullet2added:Patientswithviralhepatitiswhohavehadacompleteorsustainedviralresponseshouldcontinuewithscreeningdespitethatresponse.HCC-A2of3Footnoteaadded:Theoptimaldiagnosticmethodiscoreneedlebiopsy.SeePrinciplesofCoreNeedleBiopsy(HCC-B).HCC-A(3of3)•Reference5added:Ioannou,GN.HCCsurveillanceafterSVRinpatientswithF3/F4fibrosis.JHepatol2021;74:458-465.•Reference11updated:ClaudonM,DietrichCF,ChoiBI,etal.Guidelinesandgoodclinicalpracticerecommendationsforcontrastenhancedltrasound(CEUS)intheliver-update2012:AWFUMB-EFSUMBinitiativeincooperationwithrepresentativesofAFSUMB,AIUM,ASUM,FLAUS.UltrasoundMedBiol2013;4639:187-210.DietrichCF,NolsøeCP,BarrRG,etal.Guidelinesandgoodclinicalpracticerecommendationsforcontrast-enhancedultrasound(CEUS)intheliver-update2020:WFUMBincooperationwithEFSUMB,AFSUMB,AIUM,andFLAUS.UltrasoundMedBiol2020;46:2579-2604.PrintedbyMinTangon4/1/202211:01:21AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.dexersionoftheNCCNGuidelinesforHepatobiliaryCancersfromVersioninclude•Titlechanged:PrinciplesofCoreNeedleBiopsy.pAdded"Coreneedle"beforeeachinstanceofbiopsy.pFirststatementrevised:Theoptimaldiagnosticmethodiscoreneedlebiopsy.Indicatorsforconsiderationofcoreneedlebiopsymayinclude...pBullet2added:Ifcoreneedlebiopsyisconsidered,obtainpriortoablation.•PrinciplesofSurgerypBullet5added:Selectpatientswithunresectablediseasewithresponsecanbeconsideredforsurgery.Consultationwithamedicaloncologistandamultidisciplinaryteamisrecommendedtodeterminethetimingofsurgeryaftersystemictherapy.pFootnote3updated:Heimbach,JK.EvolutionofLiverTransplantSelectionCriteriaandU.S.AllocationPolicyforPatientswithHepatocellularCarcinoma,SeminLiverDis(2020)[Epubaheadofprint].HeimbachJK.EvolutionoflivertransplantselectioncriteriaandU.S.allocationpolicyforpatientswithhepatocellularcarcinoma.SeminLiverDis2020;40:358-364.HCC-F(1of2)•PrinciplesofRadiationTherapypTreatmentModalities◊Sub-bullet8revised:PalliativeEBRTisappropriateforsymptomcontroland/orpreventionofcomplicationsfrommetastaticHCClesions,suchasboneorbrain,andextensivelivertumorburden.pBullet2revised,RTDosing,dependingontheabilitytomeetnormalorganconstraintsandunderlyingliverfunction:◊Sub-bullet1revised:EBRT:SBRTorHypofractionationpreferred.◊Tertiarybullet1revised:SBRT:30–50Gy(typicallyin3–5fractions),dependingontheabilitytomeetnormalorganconstraintsandunderlyingliverfunction.◊Tertiarysub-bulletsadded:–Hypofractionation▪37.5–72Gyin10–15fractions.–Conventionalfractionation:▪50–66GYin25–33fractions.HCC-F(2of2)•PrinciplesofRadiationTherapy,ReferencespReferenceremoved:TaoR,KrishnanS,BhosalePR,etal.Ablativeradiotherapydosesleadtoasubstantialprolongationofsurvivalinpatientswithinoperableintrahepaticcholangiocarcinoma:aretrospectivedoseresponseanalysis.JClinOncol2016;34:219-226.pReference1added:ApisarnthanaraxS,BarryA,CaoM,etal.Externalbeamradiationtherapyforprimarylivercancers:AnASTROClinicalPracticeGuideline.PractRadiatOncol2022;12:28-51.liativeradiotherapyforhepatocellularcarcinomaandlivermetastasesJClinOncol2013;31:3980-3986.iontherapyforlivertumorsIntJRadiatOncolBiolPhys1;110:188-195.pReference13added:SeongJ,LeeIJ,ShimSJ,etal.AmulticenterretrospectivecohortstudyofpracticepatternsandclinicaloutcomeonradiotherapyforhepatocellularcarcinomainKorea.LiverInt2009;29:147-152.pReference14added:Ben-JosefE,LawrenceTS.Radiotherapyforunresectablehepaticmalignancies.SeminRadiatOncol2005;15:273-278.UPDATESVersion1.2022,03/29/22©2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.PrintedbyMinTangon4/1/202211:01:21AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.dexersionoftheNCCNGuidelinesforHepatobiliaryCancersfromVersioninclude•First-LineSystemicTherapypOtherRecommendedRegimens:DurvalumabaddedasaCategory2Arecommendation.pOtherRecommendedRegimens:PembrolizumabaddedasaCategory2Brecommendation.pUsefulinCertainCircumstances:RemovedFOLFOX(category2B).•Subsequent-LineTherapyIfDiseaseProgressionpBullet3revised:Ramucirumab(AFP≥400ng/mLandChild-PughClassAonly)(category1).espFootnoteerevised:"Caution:TherearelimitedsafetydataavailableforpatientswithChild-PughClassBorCliverfunctionpatientsanddosingisuncertain..."pFootnotegrevised:Therearenocomparativedatatodefineoptimaltreatmentafterfirst-linesystemictherapy.Therearenodatatodefineoptimaltreatmentforthosewhoprogressafterfirst-linesystemictherapy,otherthansorafenibornivolumab.pFootnoteiadded:Thedatareflectuseonoraftersorafenib.pFootnoteremoved:TherearelimiteddatasupportingtheuseofFOLFOX,anduseofchemotherapyinthecontextofaclinicaltrialispreferred.(QinS,etal.JClinOncol2013;31:3501-3508).HCC-G(2of2)•Reference5revised:AlsinaA,KudoM,VogelA,etal.Subsequentanticancermedicationfollowingfirst-linelenvatinib:aposthocresponderanalysisfromthephase3REFLECTstudyinunresectablehepatocellularcarcinoma.JClinOncol2019;37:371-371.AlsinaA,KudoM,VogelA,etal.Effectsofsubsequentsystemicanticancermedicationfollowingfirst-linelenvatinib:Aposthocresponderanalysisfromthephase3REFLECTstudyinunresectablehepatocellularcarcinoma.LiverCancer2020;9:93-104.•Reference6added:Abou-AlfaGK,ChanSL,KudoM,etal.Phase3randomized,open-label,multicenterstudyoftremelimumab(T)anddurvalumab(D)asfirst-linetherapyinpatients(pts)withunresectablehepatocellularcarcinoma(uHCC):HIMALAYA[abstract].JClinOncol2022;40:Abstract379.•Reference7added:vanLaethemJL,BorbathI,KarwalM,etal.Updatedresultsforpembrolizumab(pembro)monotherapyasfirst-linetherapyforadvancedhepatocellularcarcinoma(HCC)inthephaseIIKEYNOTE-224study[abstract].AnnOncol2021;32:Abstract933P.•Reference8revised:YauT,ParkJW,FinnRS,etal.CheckMate459:arandomized,multi-centerphaseIIIstudyofnivolumab(NIVO)vssorafenib(SOR)asfirst-line(1L)treatmentinpatients(pts)withadvancedhepatocellularcarcinoma.AnnOncol2019Oct;30Suppl5:v874-v87.YauT,ParkJW,FinnRS,etal.Nivolumabversussorafenibinadvancedhepatocellularcarcinoma(CheckMate459):Arandomised,multicentre,open-label,phase3trial.LancetOncol2022;23:77-90.•Reference11revised:ZhuAX,KangYK,YenCJ,etal.REACH-2:Arandomized,double-blind,placebo-controlledphase3studyoframucirumabversusplaceboassecond-linetreatmentinpatientswithadvancedhepatocellularcarcinoma(HCC)andelevatedbaselinealpha-fetoprotein(AFP)followingfirst-linesorafenib.JClinOncol2018;36:4003.ZhuAX,KangYK,YenCJ,etal.Ramucirumabaftersorafenibinpatientswithadvancedhepatocellularcarcinomaandincreasedα-fetoproteinconcentrations(REACH-2):Arandomised,double-blind,placebo-controlled,phase3trial.LancetOncol2019;20:282-296.•Reference14added:FinnRS,RyooB-Y,MerleP,etal.Pembrolizumabassecond-linetherapyinpatientswithadvancedhepatocellularcarcinomainKEYNOTE-240:Arandomized,double-blind,phaseIIItrial.JClinOncol2020;38:193-202.•Reference15added:QinS,ChenZ,FangW,etal.Pembrolizumabplusbestsupportivecareversusplaceboplusbestsupportivecareassecond-linetherapyinpatientsinAsiawithadvancedhepatocellularcarcinoma(HCC):KEYNOTEstudy[abstract].JClinOncol2022;40:Abstract383.•Reference17revised:KudoM,MatillaA,SantoroA,etal.Checkmate-040cohort5:AphaseI/IIstudyofnivolumabinpatientswithadvancedhepatocellularcarcinomaandChild-PughBcirrhosis.JHepatol2021;75:600-609nivolumab(NIVO)inpatients(pts)withadvancedhepatocellularcarcinoma(aHCC)andChild-PughB(CPB)status.JClinOncol2019;37:327-327.•Reference18added:MatillaA,SangroB,El-KhoureiryA,etal.Checkmate040cohort5:Long-termefficacyandsafetyofnivolumabinpatientswithChild-PughBadvancedhepatocellularcarcinoma:Associationsbetweenbaselinebiomarkeranalysesandoutcomes[abstract].JHepatol2021:75:AbstractOS-295.UPDATESVersion1.2022,03/29/22©2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.PrintedbyMinTangon4/1/202211:01:21AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.dexersionoftheNCCNGuidelinesforHepatobiliaryCancersfromVersionincludeBiliaryTractCancersGallbladderCancerThissectionhasbeensignificantlyrevised.Newalgorithm:Hepatobiliarysurgeryexpertiseunavailable.tionpT1borgreaterand/orT1awithpositivemargins.•PrimaryTreatmentpUnresectable◊"TMBtesting"sub-bulletmovedaboveAdditionalmoleculartestingasabullet.(AlsoforGALL-4,GALL-5,INTRA-1,EXTRA-1)esorGALLGALLaddedTheoptimaldiagnosticmethodiscoreneedlebiopsyAlsoforGALL•PresentationandWorkuppMetastaticdisease◊"TMBtesting"sub-bulletmovedaboveAdditionalmoleculartestingasabullet.(AlsoforINTRA-1,EXTRA-1)•Treatmentp"Options"addedabovethetreatmentoptions.pResected,negativemargin(R0),negativeregionalnodes,carcinomainsituatmarginpathway,bulletorderhasbeenrevised.pResectedgrossresidualdisease(R2)pathway,revised:Seetreatmentforunresectabledisease.•Footnotehadded:Orderdoesnotindicatepreference.Thechoiceoftreatmentmodalitymaydependonextent/locationofdiseaseandinstitutionalcapabilities.GALL-A(1of2)•PrinciplesofSurgeryandPathologypIncidentalFindingatSurgery◊Bullet2revised:Ifexpertiseisavailableandthereisconvincingclinicalevidenceofcancer,adefinitiveresectioncanshouldbeperformedaswrittenbelow.Ifthediagnosisisnotclear,frozensectionbiopsiescanbeconsideredinselectedcasesbeforeproceedingwithdefinitiveresection.Ifmalignancyissuspectedorconfirmedaftercholecystectomyhasbeeninitiatedandexpertiseisavailable,thendefinitiveresectionshouldbeundertaken.◊Bullet3added:Ifmalignancyissuspectedbeforecholecystectomyhasbegunandthereisaquestionofresectability(ie,locallyadvanced,possiblemetastaticdisease,other),thendefinitiveresectioncanbepostponed,regardlessofavailableexpertise,untilcompletestagingandevaluationhasbeenperformed.Documentallfindingsandconsiderbiopsyifchemotherapyisanticipated.pFootnoteaadded:Theoptimaldiagnosticmethodiscoreneedlebiopsy.Version1.2022,03/29/22©2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.UPDATESPrintedbyMinTangon4/1/202211:01:21AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.dexersionoftheNCCNGuidelinesforHepatobiliaryCancersfromVersionincludegiocarcinomaablepRecommendationadded:Biopsy,ifnotpreviouslyperformed.(AlsoforMetastaticdisease)•PrimaryTreatmentpMetastaticdiseasepathway,bullet1revised:Systemictherapy(preferred).pMetastaticdiseasepathway,bullet2revised:Clinicaltrial(preferred).•Footnotebrevised:SeePrinciplesofImaging(HCC-A)(BIL-A).(AlsoforINTRA-2)•Footnotefadded:Theoptimaldiagnosticmethodiscoreneedlebiopsy.•Footnotenrevised:Intra-arterialchemotherapy(withorwithoutsystemicchemotherapy)maybeusedinaclinicaltrialoratexperiencedcentersincarefullyselectedcases.•Footnotepadded:SeeNCCNGuidelinesforPalliativeCare.•TreatmentpNoresiduallocaldisease(R0resection),bulletorderhasbeenrevised.◊Bullet1,revised:Systemictherapy(preferred).◊Bullet2,revised:Clinicaltrial(preferred).pMicroscopicmargins(R1)orPositiveregionalnodes,bulletorderhasbeenrevised.◊Bullet1,revised:Systemictherapy(preferred).◊Bullet2,revised:Clinicaltrial(preferred).pResiduallocaldisease(R2resection)pathwayrevised:Seetreatmentforunresectabledisease.ExtrahepaticCholangiocarcinoma•Footnotehrevised:Considerbiliarydrainageforpatientswithjaundicepriortoinstitutingsystemictherapychemotherapy.ConsiderbaselineCA19-9afterbiliarydecompression.•Footnoteiadded:Theoptimaldiagnosticmethodiscoreneedlebiopsy.•Footnotepadded:SeeNCCNGuidelinesforPalliativeCare.EXTRA-2•TreatmentpResected,negativemargin(R0),NegativeregionalnodesorCarcinomainsituatmargin,bulletorderhasbeenrevised.◊"Options"addedabovethetreatmentoptions.◊Bullet1revised:Systemictherapy(preferred).◊Bullet2revised:Clinicaltrial(preferred).pResected,positivemargin(R1)orPositiveregionalnodes,bulletorderhasbeenrevised.◊"Options"addedabovethetreatmentoptions.◊Bullet1revised:Systemictherapy(preferred).◊Bullet2revised:Clinicaltrial(preferred).pResectedgrossresidualdisease(R2)pathwayrevised:SeetreatmentforunresectablediseasepFootnotesadded:Orderdoesnotindicatepreference.Thechoiceoftreatmentmodalitymaydependonextent/locationofdiseaseandinstitutionalcapabilities.Version1.2022,03/29/22©2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.UPDATESPrintedbyMinTangon4/1/202211:01:21AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.dexersionoftheNCCNGuidelinesforHepatobiliaryCancersfromVersionincludepReference6added:SuttonTL,BillingsleyKG,WalkerBS,etal.Detectionoftumormultifocalityinresectableintrahepaticcholangiocarcinoma:Definingtheoptimalpre-operativeimagingmodality.JGastrointestSurg2021;25:2250-2257.pReference7added:ACR-SAR-SPRpracticeparameterfortheperfor