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?TMemorialSloanKetteringCancerCenter*StephenAnsell,MD,PhD/Vice-Chair?MayoClinicCancerCenterWeiyunZ.Ai,MD,PhD??UCSFHelenDillerFamilyComprehensiveCancerCenterJeffreyBarnes,MD,PhD?MassachusettsGeneralHospitalCancerCenterStefanK.Barta,MD,MRCP,MS??AbramsonCancerCenterheUniversityofPennsylvaniaJonathanBrammer,MD?TheOhioStateUniversityComprehensiveCancerCenter-JamesCancerHospitalandSoloveResearchInstituteMarkW.Clemens,MD?TheUniversityofTexasAhmetDogan,MD,PhD≠M(fèi)emorialSloanKetteringCancerCenterFrancineFoss,MD??ξYaleCancerCenter/SmilowCancerHospitalPaolaGhione,MD?RoswellParkComprehensiveCancerCenterAaronM.Goodman,MD?ξUCSanDiegoMooresCancerCenterJoanGuitart,MD≠?RobertH.LurieComprehensiveCancerCenterofNorthwesternUniversityesPanelDisclosuresAhmadHalwani,MD?HuntsmanCancerInstituteattheUniversityofUtahBradleyM.Haverkos,MD,MPH,MS?UniversityofColoradoCancerCenterRichardT.Hoppe,MD§StanfordCancerInstituteEricJacobsen,MD?Dana-Farber/BrighamandWomen'senterDeepaJagadeesh,MD,MPH??CaseComprehensiveCancerCenter/UniversityHospitalsSeidmanCancerCenterandClevelandClinicTaussignstituteAllisonJones,MD?St.JudeChildren'sResearchHospital/TheUniversityofTennesseeHealthScienceCenterAvyaktaKallam,MD,MBBSTFred&PamelaBuffettCancerCenterYounH.Kim,MD??StanfordCancerInstituteKiranKumar,MD,MBA§UTSouthwesternSimmonsComprehensiveCancerCenterNehaMehta-Shah,MD,MSCI??SitemanCancerCenteratBarnes-JewishHospitalandWashingtonUniversitySchoolofMedicineEliseA.Olsen,MD??DukeCancerInstituteSaurabhA.Rajguru,MD??UniversityofWisconsinCarboneCancerCenterSimaRozati,MD,PhD?TheSidneyKimmelComprehensiveCancerCenteratJohnsHopkinsJonathanSaid,MD≠UCLAJonssonComprehensiveCancerCenterAaronShaver,MD,PhD≠Vanderbilt-IngramCancerCenterLaurenShea,MD?O'NealComprehensiveCancerCenteratUABMichiM.Shinohara,MD?≠FredHutchinsonCancerResearchCenter/SeattleCancerCareAllianceLubomirSokol,MD,PhD??TMoffittCancerCenterCarlosTorres-Cabala,MD≠TheUniversityofTexasMDAndersonCancerCenterRyanWilcox,MD,PhD??UniversityofMichiganRogelCancerCenterPeggyWu,MD,MPH?UCDavisComprehensiveCancerCenterJasmineZain,MD??CityofHopeNationalMedicalCenterξBonemarrowtransplantation?Dermatology?Hematology/HematologyoncologyTInternalmedicine?Medicaloncology≠Pathology?Plasticsurgery§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,TCR?,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