原发皮肤淋巴瘤2022.v1（英文）-NCCN肿瘤临床实践指南

NCCNClinicalPracticeGuidelinesinOncologyNCCNGuidelines®)PrimaryCutaneousLymphomaersionJanuaryVersion1.2022,01/26/22©2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.PrintedbyMinTangon3/14/20227:57:50AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.dex*StevenM.Horwitz,MD/Chair†ÞMemorialSloanKetteringCancerCenter*StephenAnsell,MD,PhD/Vice-Chair‡MayoClinicCancerCenterWeiyunZ.Ai,MD,PhD†‡UCSFHelenDillerFamilyComprehensiveCancerCenterJeffreyBarnes,MD,PhD†MassachusettsGeneralHospitalCancerCenterStefanK.Barta,MD,MRCP,MS†‡AbramsonCancerCenterheUniversityofPennsylvaniaJonathanBrammer,MD‡TheOhioStateUniversityComprehensiveCancerCenter-JamesCancerHospitalandSoloveResearchInstituteMarkW.Clemens,MDʘTheUniversityofTexasAhmetDogan,MD,PhD≠MemorialSloanKetteringCancerCenterFrancineFoss,MD†‡ξYaleCancerCenter/SmilowCancerHospitalPaolaGhione,MD‡RoswellParkComprehensiveCancerCenterAaronM.Goodman,MD‡ξUCSanDiegoMooresCancerCenterJoanGuitart,MD≠ϖRobertH.LurieComprehensiveCancerCenterofNorthwesternUniversityesPanelDisclosuresAhmadHalwani,MD‡HuntsmanCancerInstituteattheUniversityofUtahBradleyM.Haverkos,MD,MPH,MS†UniversityofColoradoCancerCenterRichardT.Hoppe,MD§StanfordCancerInstituteEricJacobsen,MD†Dana-Farber/BrighamandWomen'senterDeepaJagadeesh,MD,MPH†‡CaseComprehensiveCancerCenter/UniversityHospitalsSeidmanCancerCenterandClevelandClinicTaussignstituteAllisonJones,MDϖSt.JudeChildren'sResearchHospital/TheUniversityofTennesseeHealthScienceCenterAvyaktaKallam,MD,MBBSÞFred&PamelaBuffettCancerCenterYounH.Kim,MDϖ†StanfordCancerInstituteKiranKumar,MD,MBA§UTSouthwesternSimmonsComprehensiveCancerCenterNehaMehta-Shah,MD,MSCI†‡SitemanCancerCenteratBarnes-JewishHospitalandWashingtonUniversitySchoolofMedicineEliseA.Olsen,MDϖ†DukeCancerInstituteSaurabhA.Rajguru,MD†‡UniversityofWisconsinCarboneCancerCenterSimaRozati,MD,PhDϖTheSidneyKimmelComprehensiveCancerCenteratJohnsHopkinsJonathanSaid,MD≠UCLAJonssonComprehensiveCancerCenterAaronShaver,MD,PhD≠Vanderbilt-IngramCancerCenterLaurenShea,MD‡O'NealComprehensiveCancerCenteratUABMichiM.Shinohara,MDϖ≠FredHutchinsonCancerResearchCenter/SeattleCancerCareAllianceLubomirSokol,MD,PhD†‡ÞMoffittCancerCenterCarlosTorres-Cabala,MD≠TheUniversityofTexasMDAndersonCancerCenterRyanWilcox,MD,PhD†‡UniversityofMichiganRogelCancerCenterPeggyWu,MD,MPHϖUCDavisComprehensiveCancerCenterJasmineZain,MD†‡CityofHopeNationalMedicalCenterξBonemarrowtransplantationϖDermatology‡Hematology/HematologyoncologyÞInternalmedicine†Medicaloncology≠PathologyʘPlasticsurgery§Radiotherapy/Radiationoncology*DiscussionWritingteeMemberVersion1.2022,01/26/22©2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.PrintedbyMinTangon3/14/20227:57:50AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.dexymphomasPanelMembersaryoftheGuidelinesUpdatesPrimaryCutaneousB-CellLymphomas•DiagnosisandWorkup(CUTB-1)•PrimaryCutaneousMarginalZoneLymphoma(CUTB-2)•PrimaryCutaneousFollicleCenterLymphoma(CUTB-2)•TNMClassificationofCutaneousLymphomaotherthanMF/SS(CUTB-A)•TreatmentReferences(CUTB-B)MycosisFungoides/SézarySyndrome(MF/SS)•OverviewofDefinitionandDiagnosis(MFSS/INTRO-1)•GeneralPrinciples(MFSS/INTRO-2)•Diagnosis(MFSS-1)•Workup(MFSS-2)•TNMBClassificationandStaging(MFSS-3)•ClinicalStaging(MFSS-4)•StageIA(LimitedSkinInvolvementAlone,<10%BSA)(MFSS-6)•StageIB(SkinOnlyDiseasewith≥10%BSA)-StageIIA(MFSS-7)•StageIIB(TumorStageDisease)(MFSS-8)•StageIII(ErythrodermicDisease)(MFSS-10)•StageIV(MFSS-11)•LargeCellTransformation(LCT)(MFSS-12)•SuggestedTreatmentRegimens(MFSS-A)•SupportiveCare(MFSS-B)PrimaryCutaneousCD30+T-CellLymphoproliferativeDisorders•OverviewandDefinition(PCTLD/INTRO-1)•Diagnosis(PCTLD-1)•Workup(PCTLD-2)•PrimaryCutaneousALCL(PCTLD-3)•LymphomatoidPapulosis(PCTLD-4)•TherapyReferences(PCTLD-A)YMAtiveCareforPatientsWithCutaneousCnDifferentialDiagnosisofMatureBCellandmsSeeNCCNGuidelinesBCellLymphomasNHODGAsDiffuseLargeBCellelinesrBCellLymphomasDLBCLlievesthatthebestmanagementforanypatientwithcancerisinaclinicaltrial.Participationinclinicaltrialsisespeciallyencouraged.FindanNCCNMemberInstitution:/home/member-institutions.ofEvidenceandendationsssotherwiseedCCNCategoriesofEvidenceandConsensus.NCCNCategoriesofPreference:Allrecommendationsareconsideredappropriate.SeeNCCNCategoriesofPreference.TheNCCNGuidelinesareastatementofevidenceandconsensusoftheauthorsregardingtheirviewsofcurrentlyacceptedapproachestotreatmentAnyclinicianseekingtoapplyorconsulttheNCCNGuidelinesisexpectedtouseindependentmedicaljudgmentinthecontextofindividualstancestodetermineanypatientscareortreatmentTheNationalComprehensiveCancerNetworkNCCNmakesnorepresentationsorwarrantiesofanykindregardingtheircontentuseorapplicationanddisclaimsanyresponsibilityfortheirapplicationoruseinanywayTheNCCNbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.©2022.Version1.2022,01/26/22©2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.RegimenswereextensivelyreorganizedandclarifiedbypSeparatingtheregimensbyStageRegimenswereextensivelyreorganizedandclarifiedbypSeparatingtheregimensbyStageandprovidingTreatmentConsiderationsforeachstagepCombinationtherapiesmovedarenowlistedwitheachofthestagespecificrecommendationstagesMFSS-A1of12newpageGeneralConsiderationsfortheTreatmentofPatientswithdexUpdatesinVersion1.2022oftheNCCNGuidelinesforPrimaryCutaneousLymphomasfromVersion2.2021include:ughughMFSSgorithmsrevised•Suggestedtreatmentregimenreferenceswereupdatedthroughouttheguidelines.angedtotreatmentandangedtotreatmentandresponseassessmentanddirectedtotheappropriatetablesonMFSS-A.dednewpageanddirectedtotheappropriatetablesonMFSS-A.FootnoteurevisedFootnoteurevisedbyaddingGeneralConsiderationsfortheTreatmentofPatientswithMFandSSMFSSA1of12).es•Diagnosis,Usefulp1stbullet,2ndsub-bulletrevised:AssessmentofIgM,IgD,IgA,IgG,IgE,...•Footnotecadded:Amultidisciplinaryteamapproachinvolvinghematology/oncology,dermatology,pathology(withexpertiseincutaneouslymphoma),andradiationoncologyisoftenoptimalforthemanagementofpatientswithCBCL.•Solitary/Regional,Initialtherapy,"Excision"wasmovedtoinselectedcases.•Footnotegrevised:...orC/A/PCTwithcontrastattheendoftreatmentaremaybeneededtoassessresponse.(alsoforCUTB-3)•Footnoteladded:Smalllesionsmaybeexcisedwithminimalnon-disfiguringsurgery.(alsoforPCTLD-3)◊Brentuximabvedotin◊Mogamulizumab◊Romidepsin◊Brentuximabvedotin◊Mogamulizumab◊Romidepsin◊VorinostatpRemovedthefollowingsystemictherapyoptionsforStageIAMFwithbloodinvolvement◊Gemcitabine◊Liposomaldoxorubicin◊Pralatrexate◊Alemtuzumab◊Pembrolizumab•GeneralPrinciplesp1stbulletrevised:...dermatology,pathologywithexpertiseincutaneouslymphoma,and...p8thbulletrevised:Generally,skin-directedtherapiesandbiologicagentswithlowerratesofimmunosuppressionsystemictherapyregimensthatcanbetoleratedforlongerdurationsoftherapywithlowerratesofcumulativetoxicity,lessimmunosuppression,and/orhigherefficacyareusedinearlierlinesoftherapy.p9thbulletrevised:Whenchemotherapyisrequired,ingeneral,singleForStageIBIIAMFwithbloodForStageIBIIAMFwithbloodinvolvementthefollowingregimenschangedfromacategory2Atoacategory2Brecommendation.toshort-livedresponsesassociatedwithshorterdurationsoftherapyandhighertoxicityprofilesassociatedwithmultiagentregimens.Inpatientsrequiringchemotherapy,singleagentsarepreferredovercombinationchemotherapy,duetothehighertoxicityprofilesassociatedwithmulti-agentregimensandtheshort-livedresponsesseenwithtime-limitedcombinationchemotherapy."pPralatrexateUPDATESUPDATESVersion1.2022,Version1.2022,01/26/22©2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.UPDATESVersion1.2022,01/26/22©2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.PrintedbyMinTangon3/14/20227:57:50AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.dexnoftheNCCNGuidelinesforPrimaryCutaneousLymphomasfromVersionincludeMycosisFungoides/SézarySyndromeMFSS-A6of12•StageIIIMF(erythrodermicdisease)pAddedthefollowingasskin-directedtherapyoptions◊Phototherapyasacategory2Arecommendation◊TSEBTasacategory2BrecommendationMFSS-A8of12•Non-Sézary(stageIVA2)orVisceral/solidorgan(stageIVB)diseasepAddedmogamulizumabasacategory2A,OtherRecommendedRegimen•Relapsedorrefractorydiseasetomultiplepriortherapiesp7thbulletrevised:TemozolomideforCNSinvolvementatsomeNCCNMembertionsMFSS-B1of2•Pruritis,treatment,systemicagentspFirst-lineadded:pregabalinpThird-lineadded:systemicsteroidsectionspUlceratedandnecrotictumors,sub-bulletrevisedbyadding:Ulcerwillnothealunlessdiseaseistreated.ConsiderRTbeamiffeasible.LymphoproliferativeDisorders•Diagnosis,Usefulp1stbulletrevised:...perforin,GM1,IRF4/MUM1,EMA,TCRꞵ,TCRδ•CutaneousALCLWorkup,UsefulpBulletmovedfromessential:Bonemarrowaspirationandbiopsy...p2ndbulletrevisedbyadding:ifcontemplatingtreatmentsthatarecontraindicatedinpregnancy.(alsoforLyP)FootnotesaddedSmalllesionsmaybeexcisedwithminimalnon-ringsurgeryFootnotezaddedNBUVBisgenerallypreferredoverPUVA.PrinciplesofRadiationTherapyPCLYM-A1of3•Linkadded:SeeNCCNGuidelinesforHodgkinLymphoma-RadiationDoseConstraints.ortiveCare•AdverseeventsassociatedwithmogamulizumabpAdded:DrugEruption:MogamulizumabhasbeenassociatedwithadrugeruptionthatcanclinicallymimicCTCL.Skinbiopsyisrecommendedtodistinguishprogressionofdiseaseversusdrugeruption(ChenL,etalJAMADermatology2019;155:968-971;HirotsuK,etalJAMADermatol2021;157:700-707).•Typicalclinicalpresentationisredtobluishplaquesortumorslocatedononeorbothlegsthatcanulcerate.•Itisusuallyaggressiveand•Typicalclinicalpresentationisredtobluishplaquesortumorslocatedononeorbothlegsthatcanulcerate.•Itisusuallyaggressiveandassociatedwithapoorprognosis(highfrequencyofextracutaneousrelapses)(5-yearOSrateis50%).3,4•Multipleskinlesions,inactivationofCDKN2A,andMYD88L265Passociatedwithinferiorprognosis.CUTB/INTRO-1•MostcommonsubtypeofPCBCL(57%),1,2locatedprimarilyinthescalp,face,forehead,andtrunk,usuallywithindolentcourseand•Typicallypresentsassolitary,firm,andpinktoviolaceouspapules,nodules,plaques,ortumors.Multifocalskinlesionsareseenin15%•Relapsesintheskinoccurin50%ofpatients.marycutaneousdiffuselargeBcelllymphomaPCDLBCLlegtypeexcellentprognosis(5-yearoverallsurvival[OS]rateis>95%).erarestsubtypeofPCBCLconstitutingofallprimarycutaneouslymphomasItisdistributedmostlytothelegbutdexiewDefinitionPRINCIPLESFORPRIMARYCUTANEOUSB-CELLiewDefinitioneousfollicleeousfolliclecenterlymphomaPCFCLofcases.1,2Ulcerationisrare.•Disseminationtoextracutaneoussitesisextremelyuncommon;cutaneousrecurrencesoccurneartheinitialsiteinofcases.1,2Ulcerationisrare.•Disseminationtoextracutaneoussitesisextremelyuncommon;cutaneousrecurrencesoccurneartheinitialsiteinapproximately30%ofcases.PrimarycutaneousmarginalzonelymphomaPCMZL•SecondmostcommonsubtypeofPCBCL(24%–31%)1,2withdistributionprimarilyonthetrunk,upperextremities,andhead.Typicallyexcellentprognosis(5-yearsurvivalrateis99%).Diagnosis•PCFCL:punchbiopsy/incision/excisionofskinlesionpreferredtoshavebiopsypImmunophenotype–cellsexpressCD20,CD79a,andBCL6;surfaceIgisnegative.CD10canbenegativeincaseswithdiffusegrowthpattern.BCL2isusuallynegative,orminimallyexpressed.pWhenCD10andBCL2arestronglyexpressed,orBCL2isrearranged,consideranodalFLwithsecondaryskininvolvement.•PCMZL:punchbiopsy/incision/excisionofskinlesionpreferredtoshavebiopsypImmunophenotype–cellsarenegativeforCD10andBCL6,butareoftenpositiveforBCL2.IgG4canbeexpressedinaboutathirdofcases.pCanbedividedinto2groupswithdifferentprognosisbasedontheIgheavychainrearrangement:1)CXCR3-negativeandIgclass-switchedsubtype(IgG,IgA,andIgE),characterizedbynodularinfiltratesofplasmacells;and2)alesscommonsubtypethatisCXCR3-positiveandIgMpositive(nonclass-switched)whichmayhaveextracutaneousextension.5-8IgGclass-switchedsubtypeisaclonalchroniclymphoproliferativedisorder(LPD),withindolentcourse.8,9•PCDLBCL,legtype:punchbiopsy/incision/excisionofskinlesionpreferredtoshavebiopsypImmunophenotype–cellsexpressCD20,CD79a,monotypicimmunoglobulins,BCL2(strong),IRF/MUM1,FOXP1,andMYC.CD10stainingisusuallynegative.pGeneexpressionprofiling:PCDLBCL,legtypehasbeendemonstratedtobealwaysactivatedB-cell(ABC)subtype.GerminalcenterB-cell(GCB)subtypeshouldraiseconcernforPCFCL,eveniflargecellsarepresent.10,11pFISH:frequentlyshowstranslocationsofMYC,BCL6,andIGHgenes.Note:Allrecommendationsarecategory2Aunlessotherwiseindicated.ClinicalTrials:NCCNbelievesthatthebestmanagementofanypatientwithcancerisinaclinicaltrial.Participationinclinicaltrialsisespeciallyencouraged.Version1.2022,01/26/22©2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.CUTB/INTRO-2PrintedbyMinTangon3/14/20227:57:50AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.CUTB/INTRO-2dexPRINCIPLESFORPRIMARYCUTANEOUSB-CELLLYMPHOMAS(PCBCL)GeneralPrinciples•PCFCL,PCMZL:Ifthepathologyorclinicalpresentationisnottypical,completestagingwithchest/abdominal/pelvicCTand/orPET/CTscantoruleoutsystemicinvolvement.Low-doselocalizedradiationtherapy,topicalorintralesionalsteroids,orobservationareexcellenttreatmentoptions.•PCDLBCL,legtype:CompletestagingwithPET/CTscan.Treatwithchemoimmunotherapyandlocalizedradiationtherapy.(SeeNCCNGuidelinesforB-CellLymphomas-DLBCL)1ZinzaniPL,QuaglinoP,PimpinelliN,etal.PrognosticfactorsinprimarycutaneousB-celllymphoma:theItalianStudyGroupforCutaneousLymphomas.JClinOncol2006;24:1376-1382.2SenffNJ,HoefnagelJJ,JansenPM,etal.Reclassificationof300primarycutaneousB-celllymphomasaccordingtothenewWHO-EORTCclassificationforcutaneouslymphomas:comparisonwithpreviousclassificationsandidentificationofprognosticmarkers.JClinOncol2007;25:1581-1587.3GrangeF,BekkenkMW,WechslerJ,etal.PrognosticfactorsinprimarycutaneouslargeB-celllymphomas:aEuropeanmulticenterstudy.JClinOncol2001;19:3602-3610.4GrangeF,Beylot-BarryM,CourvilleP,etal.PrimarycutaneousdiffuselargeB-celllymphoma,legtype:clinicopathologicfeaturesandprognosticanalysisin60cases.ArchDermatol2007;143:1144-1150.5vanMaldegemF,vanDijkR,WormhoudtTA,etal.ThemajorityofcutaneousmarginalzoneB-celllymphomasexpressesclass-switchedimmunoglobulinsanddevelopsinaT-helpertype2inflammatoryenvironment.Blood2008;112:3355-3361.6EdingerJT,KantJA,SwerdlowSH.Cutaneousmarginalzonelymphomashavedistinctivefeaturesandinclude2subsets.AmJSurgPathol2010;34:1830-1841.7KogameT,TakegamiT,SakaiTR,etal.Immunohistochemicalanalysisofclass-switchedsubtypeofprimarycutaneousmarginalzonelymphomaintermsofinducibleskin-associatedlymphoidtissue.JEurAcadDermatolVenereol.2019;33:e401-e403.8CarlsenED,SwerdlowSH,CookJR,GibsonSE.Class-switchedprimarycutaneousmarginalzonelymphomasarefrequentlyIgG4-positiveandhavefeaturesdistinctfromIgM-positivecases.AmJSurgPathol2019;43:1403-1412.9GibsonSE,SwerdlowSH.HowIdiagnoseprimarycutaneousmarginalzonelymphoma.AmJClinPathol2020;154:428-449.10HoefnagelJJ,DijkmanR,BassoK,etal.DistincttypesofprimarycutaneouslargeB-celllymphomaidentifiedbygeneexpressionprofiling.Blood2005;105:3671-3678.11MenguyS,Beylot-BarryM,ParrensM,etal.PrimarycutaneouslargeB-celllymphomas:relevanceofthe2017WorldHealthOrganizationclassification:clinicopathologicalandmolecularanalysesof64cases.Histopathology2019;74:1067-1080.Note:Allrecommendationsarecategory2Aunlessotherwiseindicated.ClinicalTrials:NCCNbelievesthatthebestmanagementofanypatientwithcancerisinaclinicaltrial.Participationinclinicaltrialsisespeciallyencouraged.Version1.2022,01/26/22©2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.HepatitisBandCtestingfDiscussionoffertilityandspermbanking,iffertilityimpactingtherapyisplanned•Historyandphysicalexam,includingcompleteskinexam•CBCwithdifferential•ComprehensivemetabolicpanelHepatitisBandCtestingfDiscussionoffertilityandspermbanking,iffertilityimpactingtherapyisplanned•Historyandphysicalexam,includingcompleteskinexam•CBCwithdifferential•ComprehensivemetabolicpanelLDH•Chest/abdominal/pelvicCTwithcontrastand/orPET/CTscan(maybeomittedifclinicallyindicated)•Pregnancytestinginpatientsofchildbearingpotential(ifchemotherapyorRTplanned)USEFULINSELECTEDCASES:•Bonemarrowbiopsye•Peripheralbloodflowcytometry,ifCBCdemonstrateslymphocytosis•SPEP/quantitativeimmunoglobulinsforPCMZL•HIVtestingesnterdexDIAGNOSISaWORKUPc•ReviewofallslideswithatleastoneparaffinblockrepresentativeofthetumorshouldbedonebyapathologistwithexpertiseinthediagnosisofprimarycutaneousB-celllymphoma.Rebiopsyifconsultmaterialisnondiagnostic.•Adequatebiopsy(punch,incisional,excisional)ofclinicallesions•AdequateimmunophenotypingtoestablishdiagnosisbpIHCpanelmayinclude:CD20,CD3,CD10,BCL2, •AdditionalimmunohistochemicalstudiestoestablishlymphomasubtypeseLargeBCellPCseLargeBCellPCdelinesmphomasDLBCLCD23,cyclinD1,kappa/lambda(IHCorISH)pAssessmentofIgM,IgD,IgA,IgG,IgE,andFOXP1expression(tofurtherhelpindistinguishingPC-DLBCL,legtypefromPCFCL)EBER-ISH•Cytogenetics(FISHandkaryotype):t(14;18)ifsystemicFLissuspected•Ifadequatebiopsymaterialavailable,flowcytometryorIgHgenerearrangementstudiescanbeusefulindRuleoutdrug-dRuleoutdrug-inducedcutaneouslymphoidhyperplasia.eOftenreservedforpatientwithunexplainedcytopeniasorifthereisclinicalsuspicionofothersubtypeseOftenreservedforpatientwithunexplainedcytopeniasorifthereisclinicalsuspicionofothersubtypes(eg,PC-DLBCL,legtype).fHepatitisBtestingisindicatedbecauseoftheriskofreactivationwitherapySeemonoclonalantibodyandviralreactivationintheNCCNGuidelinesB-CellLymphomas.TestsincludehepatitisBsurfaceantigenandcoreantibodyforapatientwithnoriskfactorsForpatientswithriskfactorsorprevioushistoryofhepatitisBaddeantigen.Ifpositive,checkviralloadandconsultwithgastroenterologist.theNCCNGuidelinesforB-CellLymphomas.Agerminal(orfollicle)centerphenotypeandlargecellsinaskinlesionisnotequivalenttoDLBCLbutisconsistentwithprimarycutaneousgerminal/folliclecenterlymphoma.bSeeUseofImmunophenotyping/GeneticTestinginDifferentialDiagnosisofMatureB-CellandNK/T-CellNeoplasms(SeeNCCNGuidelinesforB-CellLymphomas).cAmultidisciplinaryteamapproachinvolvinghematology/oncology,dermatology,pathology(withexpertiseincutaneouslymphoma),andradiationoncologyisoftenoptimalforthemanagementofpatientswithPCBCL.Note:Allrecommendationsarecategory2Aunlessotherwiseindicated.ClinicalTrials:NCCNbelievesthatthebestmanagementofanypatientwithcancerisinaclinicaltrial.Participationinclinicaltrialsisespeciallyencouraged.CUTB-1Version1.2022,01/26/22©2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.PrintedbyMinTangon3/14/20227:57:50AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.dexPRIMARYCUTANEOUSMARGINALZONELYMPHOMAORFOLLICLECENTERLYMPHOMAgSTAGEhINITIALTHERAPYiRESPONSE/ADDITIONALTHERAPYGeneralizeddiseaseGeneralizeddiseaseousObservationousObservationkdisease)Solitary/regional,Solitary/regional,T1–2ddkinralizedralizeddiseaseskinonlyTCUTB)therapiesmIntralesionalsteroidseralizedeaseskinonlyTCUTB)TCUTB)BT3ForPCFCL,manageasFollicularLymphomaintheNCCNGuidelinesforHYPERLINK"/professionals/physician_gls/pdf/b-ce