神经内分泌肿瘤2022.v1-NCCN（英文版）

NCCNClinicalPracticeGuidelinesinOncologyNCCNGuidelines®)NeuroendocrineandAdrenalTumorsersionMaytientsavailableatwwwnccnorgpatientsVersion1.2022,05/23/22©2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.PrintedbyMinTangon6/4/20227:41:09AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.NCCNGuidelinesVersion1.2022NeuroendocrineandAdrenalTumorsdex*ManishaH.Shah,MD/Chair†TheOhioStateUniversityComprehensiveCancerCenter-JamesCancerHospitalandSoloveResearchInstitutefettCancerCenterhitneySfettCancerCenterAlB.Benson,III,MD†RobertH.LurieComprehensiveCancerCenterofNorthwesternUniversityEmilyBergsland,MD†UCSFHelenDillerFamilyComprehensiveCancerCenterLawrenceS.Blaszkowsky,MD‡MassachusettsGeneralHospitalCancerCenterckMSDeUniversityComprehensivecerCenterJamesCancerHospitaldSoloveResearchInstituteJenniferChan,MD†Dana-Farber/BrighamandWomen’senterSatyaDasMD,MSCI†Vanderbilt-IngramCancerCenterPaxtonV.Dickson,MD¶St.JudeChildren'sResearchHospital/TheUniversityofTennesseeHealthScienceCenterPaulFanta,MD‡†UCSanDiegoMooresCancerCenteriganiganenterThorvardurR.Halfdanarson,MD‡Þ†MayoClinicCancerCenteresPanelDisclosuresDanielHalperin,MD†TheUniversityofTexasJinHe,MD,PhD¶TheSidneyKimmelComprehensiveCancerCenteratJohnsHopkinsAnthonyHeaney,MD,PhDðUCLAJonssonComprehensiveCancerCenteryofHopeNationalMedicalCenteryofHopeNationalMedicalCenterArashKardan,MDɸCaseComprehensiveCancerCenter/UniversityHospitalsSeidmanCancerCenterandClevelandClinicTaussigCancerInstituteSyedM.Kazmi,MD†UTSouthwesternSimmonsCommprehensinveCancerCenterrSmilowrSmilowitalEdwardJ.Kim,MD,PhD†UCDavisComprehensiveCancerCenterBorisW.Kuvshinoff,II,MD,MBA¶RoswellParkComprehensiveCancerCenterofColoradoCancerCenterofColoradoCancerCenterKimberlyMiller,RN¥Fred&PamelaBuffettCancerCenterDianeReidy,MD†MemorialSloanKetteringCancerCenterJ.BartRose,MD¶O'NealComprehensiveCancerCenteratUABShaguftaShaheen,MD†StanfordCancerInstituteHeloisaP.Soares,MD,PhD†HuntsmanCancerInstituteattheUniversityofUtahMichaelC.Soulen,MD∩AbramsonCancerCenterattheUniversityofPennsylvanianterCraigR.nterCraigR.Sussman,MDðÞngramCancerCenterNikolaosA.Trikalinos,MD‡†SitemanCancerCenteratBarnes-JewishHospitalandWashingtonUniversitySchoolofMedicineonsinonsineCancerCenterNamrataVijayvergia,MD†FoxChaseCancerCenterTerenceWong,MD,PhDɸфDukeCancerInstituteDavidB.Zhen,MD†FredHutchinsonCancerResearchCenter/SeattleCancerCareAllianceMcCulloughRNBSerPhDDCancergeneticsфDiagnosticradiologyðEndocrinology‡Hematology/HematologyoncologyÞInternalmedicine∩Interventionalradiology†MedicaloncologyɸNuclearmedicine≠Pathology¥Patientadvocacy¶Surgery/Surgicaloncology*DiscussionsectioncommitteememberVersion1.2022,05/23/22©2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.PrintedbyMinTangon6/4/20227:41:09AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.NCCNGuidelinesVersion1.2022NeuroendocrineandAdrenalTumorsdexlievesthatthebestlievesthatthebestmanagementforanypatientwithcancerisinaclinicaltrial.Participationinclinicaltrialsisespeciallyencouraged.FindanNCCNMemberInstitution:/home/member-institutions.ofEvidenceanddationsotherwisedNCategoriesofEvidenceandConsensus.NCCNCategoriesofPreference:Allrecommendationsareconsideredappropriate.SeeNCCNCategoriesofPreference.SummaryoftheGuidelinesUpdatesClinicalPresentationsandDiagnosis(CP-1)NeuroendocrineTumorsoftheGastrointestinalTract(Well-DifferentiatedGrade1/2),Lung,andThymus(NET-1)NeuroendocrineTumorsofthePancreas(Well-DifferentiatedGrade1/2)(PanNET-1)NeuroendocrineTumorsofUnknownPrimary(NUP-1)Well-Differentiated,Grade3NeuroendocrineTumors(WDG3-1)ExtrapulmonaryPoorlyDifferentiatedNeuroendocrineCarcinoma/LargeorSmallCellCarcinoma/MixedNeuroendocrine-Non-NeuroendocrineNeoplasm(PDNEC-1)AdrenalGlandTumors(AGT-1)Pheochromocytoma/Paraganglioma(PHEO-1)MultipleEndocrineNeoplasia,Type1(MEN1-1)MultipleEndocrineNeoplasia,Type2(MEN2-1)PrinciplesofPathologyforDiagnosisandReportingofNeuroendocrineTumors(NE-A)PrinciplesofImaging(NE-B)PrinciplesofBiochemicalTesting(NE-C)SurgicalPrinciplesforManagementofNeuroendocrineTumors(NE-D)PrinciplesofHereditaryCancerRiskAssessmentandGeneticCounseling(NE-E)PrinciplesofSystemicAnti-TumorTherapy(NE-F)PrinciplesofPeptideReceptorRadionuclideTherapy(PRRT)with177Lu-dotatate(NE-G)PrinciplesofLiver-DirectedTherapyforNeuroendocrineTumorMetastases(NE-H)PrinciplesofHormoneControl(NE-I)TheNCCNGuidelinesareastatementofevidenceandconsensusoftheauthorsregardingtheirviewsofcurrentlyacceptedapproachestotreatmentAnyclinicianseekingtoapplyorconsulttheNCCNGuidelinesisexpectedtouseindependentmedicaljudgmentinthecontextofindividualstancestodetermineanypatientscareortreatmentTheNationalComprehensiveCancerNetworkNCCNmakesnorepresentationsorwarrantiesofanykindregardingtheircontentuseorapplicationanddisclaimsanyresponsibilityfortheirapplicationoruseinanywayTheNCCNbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.©2022.Version1.2022,05/23/22©2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.UPDATESVersion1.2022,05/23/22©2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.•SSRrevisedtoSSTR.Optionrevised,inmostinstances:octreotideLAR•Sectionheaderrevised:ExtrapulmonaryPoorlyDifferentiatedNeuroendocrineCarcinoma/LargeorSmallCellCarcinoma/MixedNeuroendocrine-Non-NeuroendocrineNeoplasm•SSRrevisedtoSSTR.Optionrevised,inmostinstances:octreotideLAR•Sectionheaderrevised:ExtrapulmonaryPoorlyDifferentiatedNeuroendocrineCarcinoma/LargeorSmallCellCarcinoma/MixedNeuroendocrine-Non-NeuroendocrineNeoplasm.•Hyperaldosteronismrevisedto:Primaryaldosteronism.•Cushingsyndromerevisedto:Hypercortisolemia.SectionheaderrevisedPrinciplesofGeneticHereditaryCancerRiskAssessmentandGeneticCounselingHereditaryEndocrineNeoplasiasNCCNGuidelinesVersion1.2022NeuroendocrineandAdrenalTumorsdexforNeuroendocrineandAdrenalTumorsfromVersionincludeGlobalNeuroendocrineTumorsoftheGastrointestinalTract(Well-DifferentiatedGrade1/2,LungandThymus)NET-3•Secondcolumn,secondpathwayrevised:Tumor>2cmorAnytumorsizewithincompleteresectionorpositivenodes/margins.•Footnotesremoved:pSeeSurgicalPrinciplesforManagementofNeuroendocrineTumors(NE-D).(AlsopageNET-4)pSomeinstitutionswillconsidermoreaggressivetreatmentsfor1-to2-cmtumorswithpoorprognosticfeatures.SeeDiscussionfordetails.•Footnotegrevised:SeeStaging(ST-6).Patientswithtumors<2cmthatdonotinvadebeyondthemesoappendixcanbeconsideredforobservation,afterpatient-physiciandiscussion.HellerD,etal.JAmCollSurg2019;228:839-851.Someinstitutionswillconsidermoreaggressivetreatmentsfor1-to2-cmtumorswithpoorprognosticfeatures.SeeDiscussionfordetails.NET-5•PrimaryTreatment/Surveillance,bottomofpage,optionrevised:RadicalresectionPartialortotalgastrectomy(basedontumorlocation)withregionallymphadenectomy(preferred)...NET-5A•Newfootnoteradded:Forsymptomand/ortumorcontrol,octreotideLAR20–30mgIMorlanreotide120mgSCevery4weeks.Foraddedsymptomcontrol,octreotide100–250mcgSCTIDcanbeconsidered.(AlsopagesNET-9,NET-10,andNET-11A)•Footnoteremoved:SeePrinciplesofSystemicAnti-TumorTherapy(NE-F).(AlsopageNET-6)NET-6•Evaluation,Locoregionaldisease(StageIIIA/B)pathway,optionrevised:LocallyuUnresectable.pBottombranchoptionrevised:PrimaryTreatmentofNon-MetastaticDiseaseoptionrevised:SeeManagementofLocoregionalAdvancedUnresectableDisease(NET-10).•Footnotevrevised:Cytotoxicchemotherapyoptionsincludeare:cisplatin+etoposideorcarboplatin+etoposide.NET-7•FourthoptionfollowingEvaluationrevised:Metastaticdisease(StageIV)orMultiplelungnodulesortumorletsandevidenceofdiffuseidiopathicpulmonaryneuroendocrinecellhyperplasia(DIPNECH).•PrimaryTherapy,bottomofpage,lastoptionrevised:SeeMetastaticDisease(NET-11).•Footnoteyrevised:Cytotoxicchemotherapyoptionsincludeare:cisplatin+etoposide,carboplatin+etoposide,ortemozolomide...•Newfootnotezadded:SeeDiscussion.UPDATESVersion1.2022,05/23/22©2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.PrintedbyMinTangon6/4/20227:41:09AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.NCCNGuidelinesVersion1.2022NeuroendocrineandAdrenalTumorsdexforNeuroendocrineandAdrenalTumorsfromVersioninclude•Surveillance,12wk–12mopost-resection,thirdbulletrevised:Abdominal±pelvicmultiphasicCTorMRIasclinicallyindicated.NET-9•Treatment,optionfollowingResectprimary+metastasesremoved:Refertosurveillanceforappropriateprimarydiseasesites(SeeNET-1throughNET-5).pNewarrowtopathway:Abdominal/pelvicmultiphasicCTorMRIevery12wk–12mo...•Lastcolumn,secondoptionrevised:PRRTwith177Lu-dotatate(ifSSTR-positiveimagingandprogressiononoctreotideLARorlanreotide)(category1forprogressivemid-guttumors).•Footnotesrevised:pFootnotegg:Resectionofasmallasymptomatic(relativelystable)primaryinthepresenceofunresectablemetastaticdiseaseisnotindicated.However,takingacarefulhistoryisrecommendedassurgerymaybeanoptionforasymptomaticpatientswithprevious,intermittentobstructionsResectionshouldbeconsideredtoreducefutureobstruction,mesentericischemia,bleedingorperforation.pFootnotejj:Ifclinicallysignificantdiseaseprogression,treatmentwithoctreotideLARorlanreotideshouldbediscontinuedfornon-functionaltumorsandcontinuedinpatientswithfunctionaltumors;thosetheseregimensmaybeusedincombinationwithanyofthesubsequentoptions...(AlsopagesNET-10,NET-11A,andNET-12)eoptionsfromprimarytherapybasedontumorgrade)...HeaderrevisedManagementofDistantMetastasesBronchopulmonaryorThymus)ORMultipleLungNodulesorTumorletsandEvidenceH•Headerrevised:TREATMENTPRIMARYTHERAPY•Lastcolumn,middleoptionrevised:Ifprogressiononfirst-linetherapy,Cconsiderchangingtoalternatefirst-linetherapyifprogressiononfirst-linetherapy.FootnotepprevisedCisplatinetoposide,carboplatin+etoposide,ortemozolomide±capecitabineCcanbeconsideredforintermediate-grade/atypicaltumorswithKi-67proliferativeindexandmitoticindexinthehigherendofthedefinedspectrum.•Surveillance,firstbulletrevised:Echocardiogramevery2-31–3yorasclinicallyindicated.•Footnoterrrevised:Forsymptomcontrol,octreotide150100–250mcgSCTIDoroctreotideLAR20–30mgIMorlanreotide120mgSCevery4weeks.Doseandfrequencymaybefurtherincreasedforsymptomcontrolasneeded.Therapeuticlevelsofoctreotidewouldnotbeexpectedtobereachedfor10–14daysafterLARinjection.Short-actingoctreotidecanbeaddedtooctreotideLARorlanreotideforrapidreliefofsymptomsorforbreakthroughsymptoms.Fordetailsontheadministrationofshort-actingand/orlong-actingoctreotidewith177Lu-dotatate,seeNE-G.•Footnotettrevised:SafetyandeEffectivenessofeverolimusinthetreatmentofpatientswithcarcinoidsyndromehavehasnotbeenestablished.UPDATESVersion1.2022,05/23/22©2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.subsequentoptions.FordetailsontheadministrationofoctreotideLARorlanreotidewith177Lu-dotatate,seeNE-G.PrintedbyMinTangon6/4/20227:41:09AM.Forpersonaluseonly.Notapprovedforsubsequentoptions.FordetailsontheadministrationofoctreotideLARorlanreotidewith177Lu-dotatate,seeNE-G.NCCNGuidelinesVersion1.2022NeuroendocrineandAdrenalTumorsdexforNeuroendocrineandAdrenalTumorsfromVersionincludeNeuroendocrineTumorsofthePancreas(Well-DifferentiatedGrade1/2)PanNET-2A•Footnoteremoved:SeePrinciplesofSystemicAnti-TumorTherapy(NE-F).(AlsopagesPanNET-4andPanNET-5)•Newfootnotenadded:Forsymptomand/ortumorcontrol,octreotideLAR20–30mgIMorlanreotide120mgSCevery4weeks.Foraddedsymptomcontrol,octreotide100–250mcgSCTIDcanbeconsidered.(AlsopagesPanNET-4andPanNET-5)PanNET-3•Footnoteprevised:SSTR-basedimagingonlyiftreatmentwithoctreotideLARorlanreotideisplanned.OctreotideLARorlanreotidecanbeconsideredbutonlyiftumorexpressesSSTRsshouldonlybegiveniftumordemonstratesSSTRs.IntheabsenceofSSTRs,octreotideLARorlanreotidecanprofoundlyworsenhypoglycemia.(SeeDiscussionfordetails).PanNET-4•ManagementofPrimaryNon-MetastaticDisease,optionfollowingDistalrevised:Distalpancreatectomy+peripancreaticlymphadenectomydissection+splenectomy.(AlsopagePanNET-5)PanNET-6•Surveillance,12wk–12mopost-resection:pThirdbulletrevised:AbdominalmultiphasicCTorMRIandchestCT(±contrast)asclinicallyindicated.pNewbulletadded:ChestCT(±contrast)asclinicallyindicated.PanNET-7•Lastcolumn,fifthoptionrevised:PRRTwith177Lu-dotatate(ifSSTR-positiveimagingandprogressiononoctreotideLARorlanreotide).PanNET-7AnonfunctionaltumorsandcontinuedinpatientswithfunctionaltumorsthosetheseregimensmaybeusedincombinationwithanyofnonfunctionaltumorsandcontinuedinpatientswithfunctionaltumorsthosetheseregimensmaybeusedincombinationwithanyoftheNeuroendocrineTumorsofUnknownPrimaryNUP-1•FollowingPrimarynotdiscoveredpathway,newoptionadded:Well-differentiatedGrade3.pNewoptionsadded:SeeManagementofWell-DifferentiatedGrade3LocoregionalDisease(WDG3-2)andLocallyAdvanced/MetastaticDisease:Favorablebiology(WDG3-3)orLocallyAdvanced/Metastaticdisease:Unfavorablebiology(WDG3-4).NeuroendocrineTumors,Well-DifferentiatedGrade3WDG3-1•Evaluation,Recommended,thirdbulletrevised:SSTR-PET/CTorSSTR-PET/MRI(inpatientswithtumorsKi≤55%).•Footnotefrevised:Therearelimitationsintermsofthedataforwhattheappropriatecutoffshouldbe,aswellasvariability/heterogeneityofKi-67inagiventumorandovertimeinserialbiopsies.Theclinicalcoursecanbeheterogeneousandtreatmentconsiderationsneedtoaccountforbothpathologicandclinicalfeaturesandhistopathologicworkupcombinedshoulddictatetherapy,notsolelyKi-67.(AlsopagesWDG3-2,WDG3-3A,andWDG3-4)WDG3-2•Newfootnotegadded:SeePrinciplesofSystemicAnti-TumorTherapy(NE-F4of9).(AlsopagesWDG3-3AandWDG3-4)•Footnotehrevised:Temozolomide±capecitabineMmayhavemoreactivityintumorsarisinginpancreascomparedtoGINETs.(AlsopageWDG3-3AandWDG3-4)UPDATESVersion1.2022,05/23/22©2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.PrintedbyMinTangon6/4/20227:41:09AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.NCCNGuidelinesVersion1.2022NeuroendocrineandAdrenalTumorsdexforNeuroendocrineandAdrenalTumorsfromVersionincludeWDG-3•TreatmentpFollowingResectablepathway,optionrevised:Resectionofprimary+metastaticsites,iffeasible,withacceptableriskandtoxicityprofile.pFollowingClinicallysignificanttumorburdenorevidenceofdiseaseprogressionpathway:◊Thirdoptionrevised:PRRTwith177Lu-dotatate(ifSSTR-positive).◊Sixthoptionrevised:Pembrolizumab(ifMSI-H,dMMR,orforTMB-Htumors([≥10mut/Mb])(category2B).•Surveillance,secondrow,Every12–24weeks(dependingontumorbiology),thirdbulletrevised:Abdominal/pelvicMRIwithcontrastorChest/aAbdominal/pelvicmultiphasicCT.WDG3-3A•Footnotekrevised:Pembrolizumabisanoptioncanbeconsideredforpatientswithmismatchrepair-deficient(dMMR),microsatelliteinstability-high(MSI-H),oradvancedtumormutationalburden-high(TMB-H)tumors(asdeterminedbyanFDA-approvedtest)thathaveprogressedfollowingpriortreatmentandhavenosatisfactoryalternativetreatmentoptions.(AlsopageWDG3-4)•Newfootnotemadded:Forsymptomand/ortumorcontrol,octreotideLAR20–30mgIMorlanreotide120mgSCevery4weeks.Foraddedsymptomcontrol,octreotide100–250mcgSCTIDcanbeconsidered.WDG3-4•Treatment,Systemictherapyoptions,fourthbulletrevised:Pembrolizumab(ifMSI-H,dMMR,orforTMB-Htumors([≥10mut/Mb]).•Surveillance,thirdbulletrevised:Abdominal/pelvicMRIwithcontrastorchest/aAbdominal/pelvicmultiphasicCT.ExtrapulmonaryPoorlyDifferentiatedNeuroendocrineCarcinoma/LargeorSmallCellCarcinoma/MixedNeuroendocrine-Non-NeuroendocrineNeoplasm•TumorType,Extrapulmonary:pSecondbulletrevised:Largeorsmallcellcarcinoma(otherthanlung).pThirdbulletrevised:Unknownprimary(poorlydifferentiated)Mixedneuroendocrine-non-neuroendocrineneoplasm.•Treatment,followingResectablepathway,bulletremoved:RTalone.•Newfootnotecadded:Poorlydifferentiatedneuroendocrinecarcinomasareoftenassociatedwithnon-neuroendocrinecomponentssuchasadenoorsquamouscellcarcinoma.Managementofthesetumorsiscontroversial.Often,chemotherapyregimensfornon-neuroendocrinecomponentsmaybeconsidered.•Footnoteerevised:Pembrolizumabcanbeconsideredforpatientswithmismatchrepair-deficient(dMMR),microsatelliteinstability-high(MSI-H),oradvancedtumormutationalburden-high(TMB-H(≥10mut/Mb)tumors(asdeterminedbyanFDA-approvedtest)thathaveprogressedfollowingpriortreatmentandhavenosatisfactoryalternativetreatmentoptions.UPDATESVersion1.2022,05/23/22©2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.PrintedbyMinTangon6/4/20227:41:09AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.NCCNGuidelinesVersion1.2022NeuroendocrineandAdrenalTumorsdexforNeuroendocrineandAdrenalTumorsfromVersionincludeAdrenalGlandTumorsAGT-1luationpFollowingMorphologicevaluationpathway,Adrenalprotocol:◊Firstbulletrevised:Non-contrastCTwithandwithoutcontrast(ifHU<+10,nofurtherimaging).–Newsub-bulletadded:If>+10HU,proceedwithcontrastCTwithwashout.◊Secondbulletrevised:MRIwithorandwithoutcontrasttodeterminesize,heterogeneity,lipidcontent(MRI),contrastwashout(CT),andmargincharacteristics.pFollowingFunctionalevaluationpathway,Biochemicalworkup(SeeNE-C)for:◊Bulletremoved:Suspectedadrenocorticalcarcinoma(ACC).◊Newbulletadded:Androgenexcess.•Footnoteremoved:SeePrinciplesofImaging(NE-B).•Footnoteerevised:Forbenign-appearinglesions,refertotheEndocrineSociety'sClinicalPracticeGuidelinesfortheTreatmentofCushing'sSyndrome(NiemanLK,etal.JClinEndocrinolMetab2015;100:2807-2831FleseriuM,etal.LancetDiabetesEndocrinol2021;9:847-875).(AlsopageAGT-3)•Newfootnotefadded:ACCcanoftentimessecretemultiplehormones.AGT-2ditionalEvaluationpOptionfollowingRuleoutpheochromocytomapathwayrevised:Considerimage-guidedneedlebiopsyifclinicalsuspicionofpheochromocytomaislow,metanephrines±catecholaminesarenormal,andtheresultswillimpactmanagement.pPrimaryaldosteronismpathway,suspectbenign,optionfollowingSurgicalcandidaterevised:Consideradrenalveinsamplingforaldosteroneandcortisol.AGT-3•TopoptionfollowingHypercortisolemiapathwayrevised:Tumor<4cmandbenign-appearinglesion.AGT-5•Treatment,followingLocoregionalunresectableorMetastaticdiseasepathway,fourthbulletrevised:Considersystemictherapypreferablyinclinicaltrial(SeePrinciplesofSystemicAnti-TumorTherapyforLocoregionalUnresectableorMetastaticAdrenocorticalTumorsCarcinoma[AGT-ANE-F6of9]).•Footnotewrevised:Monitormitotanebloodlevels.Someinstitutionsrecommendtargetlevelsof14–20mcg/mLiftolerated.Steady-statelevelsmaybereachedseveralmonthsafterinitiationofmitotane.Life-longhydrocortisone±fludrocortisonereplacementmaybeusuallyisrequiredwithmitotane.eochromocytoma•Evaluation,Asappropriate,ifmetastaticormultifocaldiseasesuspected,fifthbulletrevised:MIBGscanwithSPECT/CT.•Footnotefrevised:Bothcatecholaminesandmetanephrines/normetanephrinescanproducerepresentfalse-positiveresults(seeNE-C).•Footnotelrevised:MIBGscansarelesssensitivethanFDG-PETand68Ga-DOTATATEformetastaticandmultifocalparagangliomas/pheochromocytomas.SPECT/CTimagingofinvolvedsitesisrecommended.ObtainMIBGscanifconsideringtreatmentwithI131-MIBG.UPDATESVersion1.2022,05/23/22©2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.PrintedbyMinTangon6/4/20227:41:09AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.NCCNGuidelinesVersion1.2022NeuroendocrineandAdrenalTumorsdexforNeuroendocrineandAdrenalTumorsfromVersioninclude•Pageheaderaddedtotopleftofpage:MedicalPreparationForTreatment•PrimaryTreatment:pFollowingLocallyunresectablepathway,optionsrevised:◊Observe,ifasymptomaticorslow-growing,low-volumediseaseorForsecretingtumorsCcontinuemedicaltherapyalphablockadeforsecretingtumors,and:–Newbulletsadded:▪Clinicaltrial(preferred)or▪SSAs(octreotideLARorlanreotide)or▪Sunitinib37.5mgoncedailyor–Sixthbulletrevised:IfSSR-positiveimaging:cConsiderPRRTwith177Lu-dotatate,oroctreotideorlanreotide(ifsymptomatic)(ifSSTR-positive).pFollowingDistantmetastasespathway,optionsrevised:◊Observe,ifasymptomaticorslow-growing,low-volumediseaseorForsecretingtumorsCcontinuemedicaltherapyalphablockadeforsecretingtumors,and:–Newbulletsadded:▪SSAs(octreotideLARorlanreotide)or▪Sunitinib37.5mgoncedailyor–Firstbulletrevised:Clinicaltrial,(preferred)or–Sixthbulletrevised:IfSSR-positiveP