儿童侵袭性成熟B细胞淋巴瘤2021.v3（英文）-NCCN肿瘤临床实践指南

NCCNClinicalPracticeGuidelinesinOncologyNCCNGuidelines®)PediatricAggressiveMatureB-CellLymphomasersionMarchVersion3.2021,03/10/22©2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.PrintedbyMinTangon3/14/20227:09:12AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.NCCNGuidelinesVersion3.2021PediatricAggressiveMatureB-CellLymphomasdex*KimberlyDavies,MD/Chair€Dana-Farber/BostonChildren'sCancerdBloodDisordersCenter*MatthewBarth,MD/Vice-Chair€RoswellParkComprehensiveCancerCenterSaroArmenian,DO,MPH€CityofHopeNationalMedicalCenternthonyNAudinoMDeOhioStateUniversityComprehensivecerCenterJamesCancerHospitaldSoloveResearchInstituteDavidBloom,MDфUniversityofMichiganRogelCancerCenteretteMDrInstituteattheUniversityofUtahBrankoCuglievan,MD€TheUniversityofTexasdrensHospitaloMooresCancerCenterJamesB.Ford,DO€Fred&PamelaBuffettCancerCenterCancerCenteresPanelDisclosuresCancerResearchCenterianceRabiHanna,MD€ξCaseComprehensiveCancerCenter/UniversityHospitalsSeidmanCancerCenterandClevelandClinicTaussignstituteRobertHayashi,MD€ξSitemanCancerCenteratBarnes-JewishHospitalandWashingtonUniversitySchoolofMedicineAndreaJuditMachnitz,MD€фTheSidneyKimmelComprehensiveCancerCenteratJohnsHopkinsKellyW.Maloney,MD€Children'sHospitalofColorado/UniversityofColoradoCancerCenterDordCancerInstituteartinMDntercoMDnshensiveCancerCenterAbramsonCancerCenterheUniversityofPennsylvaniaailRoshalMDPhDloanKetteringCancerCenterSophieSong,MD,PhD≠UCLAJonssonComprehensiveCancerCenterJoannaWeinstein,MD€RobertH.LurieComprehensiveCancerCenterofNorthwesternUniversityAnaC.Xavier,MD€Children'sofAlabama/O'NealComprehensiveCancerCenteratUABCampbellPhDFreedmanCassPhDcMillianMSξBonemarrowtransplantationфDiagnosticradiology‡Hematology/HematologyoncologyÞInternalmedicine≠Pathology€Pediatriconcology*DiscussionWritingCommitteeMemberVersion3.2021,03/10/22©2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.PrintedbyMinTangon3/14/20227:09:12AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.NCCNGuidelinesVersion3.2021PediatricAggressiveMatureB-CellLymphomasdexlievesthatthebestmanagementforanylievesthatthebestmanagementforanypatientwithcancerisinaclinicaltrial.Participationinclinicaltrialsisespeciallyencouraged.FindanNCCNMemberInstitution:/home/member-institutions.ofEvidenceanddationsotherwisedNCategoriesofEvidenceandConsensus.NCCNCategoriesofPreference:Allrecommendationsareconsideredappropriate.SeeNCCNCategoriesofPreference.ryoftheGuidelinesUpdatesDiagnosis(PBCL-1)AdditionalDiagnosticTesting(PBCL-2)Workup(PBCL-3)GroupClassification(RiskGroupDefinitions)(PBCL-4)GroupAInductionTherapy/InitialTreatment(PBCL-5)GroupB(LowRisk)InductionTherapy/InitialTreatment(PBCL-6)GroupB(HighRisk)InductionTherapy/InitialTreatment(PBCL-7)GroupCInductionTherapy/InitialTreatment(PBCL-8)Surveillance/Follow-up(PBCL-9)TherapyforRelapseorRefractoryDisease(PBCL-10)athologyPBCLAfSystemicTherapyPBCLBaPBCLCSupportiveCarePBCLDTheNCCNGuidelinesareastatementofevidenceandconsensusoftheauthorsregardingtheirviewsofcurrentlyacceptedapproachestotreatmentAnyclinicianseekingtoapplyorconsulttheNCCNGuidelinesisexpectedtouseindependentmedicaljudgmentinthecontextofindividualstancestodetermineanypatientscareortreatmentTheNationalComprehensiveCancerNetworkNCCNmakesnorepresentationsorwarrantiesofanykindregardingtheircontentuseorapplicationanddisclaimsanyresponsibilityfortheirapplicationoruseinanywayTheNCCNationalComprehensiveCancerNetworkAllrightsreservedTheNCCNGuidelinesandtheillustrationshereinmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.©2022.Version3.2021,03/10/22©2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.UPDATESVersion3.2021,03/10/22©2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.PrintedbyMinTangon3/14/20227:09:12AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.NCCNGuidelinesVersion3.2021PediatricAggressiveMatureB-CellLymphomasdexGuidelinesforPediatricAggressiveMatureBCellLymphomasfromVersionincludePBCL-B(4of9)•PreferredTreatmentRegimensforGroupCpCOGANHL1131(RegimenC1/Pre-phaseCOP)◊Erratum:RemovedLeucovorin15mg/m²every12hoursfortwodosesonday2andagainonday4GuidelinesforPediatricAggressiveMatureBCellLymphomasfromVersionincludeMS-1•Thediscussionsectionwasupdatedtoreflectthechangesinthealgorithm.GuidelinesforPediatricAggressiveMatureBCellLymphomasfromVersioninclude•Diagnosis:UnderBiopsy;pSub-bullet2added:Touchpreparationsoffreshlesionaltissueshouldbeencouragedwheneverpossiblesince,ifdoneproperly,theymayrevealessentialcytologicdetailsthatmaybedifficulttodetectinsmallbiopsies(eg,smallneedlecorebiopsy).pSub-bullet3added:Acoreneedlebiopsyislessoptimalbutcanbeusedincircumstanceswhenalymphnodeortumormassisnoteasilyaccessibleforexcisionalorincisionalbiopsy.pSub-bullet4added:Coresmustbeofsufficientsizeandnumbertoallowforevaluationofmorphology,tumorarchitecture,andallnecessaryancillarystudies(immunohistochemistry[IHC],flowcytometry,karyotype,andFISHformajortranslocations,asapplicable).pSub-bulletremoved:Acoreneedlebiopsyislessoptimalbutcanbeusedincircumstanceswhenalymphnodeortumormassisnoteasilyaccessibleforexcisionalorincisionalbiopsy.Ifcorebiopsiesaretobeusedfordiagnosis,thecoresmustbeofsufficientsizeandnumbertoallowforevaluationofmorphology,tumorarchitecture,andallnecessaryancillarystudies(immunohistochemistry[IHC],flowcytometry,karyotype,andFISHformajortranslocationsasapplicable)PBCL-3•Workup:EssentialpBullet3revised:Evaluationforsignsorsymptomsofureteralorbowelobstruction.pImaging◊Bulletremoved:CTchest/abdomen/pelviscontrastorCTchest/MRIabdomenandpelvis.◊Bullet1added:Cross-sectionalscansoftheneck,chest,abdomenandpelvis.–Sub-bullet1new:NeckCTwithIVcontrastorMRIwithandwithoutcontrast(movedfromUsefulUnderCertainCircumstancesandedited)–Sub-bullet2new:ChestCTwithIVcontrast–Sub-bullet3new:AbdomenandpelvisCTwithoralandIVcontrastorMRIwithandwithoutcontrastpEchocardiogram(ECHO)ormultigatedacquisition(MUGA)scanandECG•Workup:UsefulunderCertainCircumstancespBulletremoved:MRIorCToftheneck,ifevidenceofneckdiseaseUPDATESVersion3.2021,03/10/22©2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.PrintedbyMinTangon3/14/20227:09:12AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.NCCNGuidelinesVersion3.2021PediatricAggressiveMatureB-CellLymphomasdexGuidelinesforPediatricAggressiveMatureBCellLymphomasfromVersionincludeFootnotewrevisedReassesssitesoforiginaldiseasewithradiologicstudiesasindicated(abdominalultrasound,chest/abdominalpelvicCTwithintravenousandoralcontrastand/orMRIofthehead,neck,abdomen,and/orpelvis).(SeePBCL-3)(AlsoPBCL-6).•Footnoteccrevisedandaddedbiosimilarstatement:...populationisdeemedappropriate.AnFDA-approvedbiosimilarisanappropriatesubstituteforrituximab.PBCL-7•Category1addedaftereachmentionofrituximab.(AlsoonPBCL-8)•Footnoteffadded:Theadditionofrituximabisacategory1recommendationforpatientswithhigh-riskGroupBandGroupCdisease.Minard-ColinV,etal.NEnglJMed2020:382:2207-2219.AnFDA-approvedbiosimilarisanappropriatesubstituteforrituximab.(AlsoPBCL-8)PBCL-7(continued)•Footnoteggrevised:Reassesssitesoforiginaldiseasewithradiologicstudiesasindicated[abdominalultrasound,chest/abdominal/pelvicCTwithintravenousandoralcontrast,and/orMRIofthehead,neck,abdomen,and/orpelvis](SeePCBL-3).BonemarrowandCSFstudiesshouldalsobeperformedifbonemarroworCSFwereinitiallyinvolved.(AlsoonPBCL-8,PBCL-10)PBCL-9•DiseaseSurveillance/Follow-uppBullet4revised:Routinesurveillanceimagingisnotrecommended.Consideronlyifclinicalsuspicionofrelapse:FDG-PET/CTorFDG-PET/MRIorCTchestwithIVcontrastandCTabdominal/pelviswithIVandoralcontrast.PBCL-A•Diagnosis-MorphologypBullet1revised:Touchpreparationsoffreshlesionaltissueshouldbeareencouragedscenarioswhererapidpreliminarydiagnosisisindicatedwheneverpossiblesince,ifdoneproperly,theymayrevealessentialcytologicdetailsthatmaybedifficulttodetectinsmallbiopsies(eg,smallneedlecorebiopsy).pSub-bullet1revised:"...andhaveroundnuclei,relativelycoarsechromatinthatisfinelydispersedwithmultiplesmallnucleoli,andscantmoderateamountsofdenselybasophiliccytoplasm..."pSub-bullet2removed:Cytoplasmicvacuolesarenottypicallypresent.•TissuesectionofBLandDLBCLarealsodistinctive.pBullet1revised:"...starrysky”appearanceindicativeofhighcellturnover.Mitosesandapoptoticbodiesareoftennumerous.Whileamorphologicspectrumis..."pBullet2revised:"...nuclearpleomorphismandscanttomoreabundant..."•Diagnosis-ImmunophenotypingpBullet1revised:"...expressTdTanddonotexpressCD34markersassociatedwithcellimmaturity(TdT,CD34)."pBullet2revised:"...bysurfaceorcytoplasmicimmunoglobulin..."pBullet4new:StrongexpressionofMUM1/IRF4,oftenwithBCL6andCD10positivity,shouldraiseconsiderationofthediagnosisoflargeB-celllymphoma(LBCL)withIRF4rearrangement.UPDATESVersion3.2021,03/10/22©2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.PrintedbyMinTangon3/14/20227:09:12AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.NCCNGuidelinesVersion3.2021PediatricAggressiveMatureB-CellLymphomasdexGuidelinesforPediatricAggressiveMatureBCellLymphomasfromVersionincludePBCL-B(1of9)•PreferredTreatmentRegimensforGroupApDoxorubicin:Underdoseandscheduleadded:Mayadministerdexrazoxane.Dexrazoxanedosingas10:1ratioofdexrazoxane:doxorubicin.(AlsoforPBCL-B,2of9,4of9,and6of9).pTripleITrevisedtoITmethotrexate,cytarabine,hydrocortisone..PBCL-B(2of9)•Table2pDoxorubicin:Underdoseandscheduleremoved,"asaone-hourinfusion."(AlsoonPBCL-B,4of9,PBCL-B,6of9,PBCL-7of9)Footnotecrevised:Rituximabisoptionalforpatientswithlow-riskGroupBdisease.(SeePBCL-6).Theadditionofrituximabisacategory1recommendationforpatientswithhigh-riskGroupBandGroupCdisease.Minard-ColinV,etal.NEnglJMed2020;382:2207-2219.AnFDA-approvedbiosimilarisanappropriatesubstituteforrituximab.(AlsoPBCL-3of9,4of9,5of9)PBCL-B(7of9)•PreferredTreatmentRegimensforRelapsed/RefractoryDiseasepNewrowadded:ITmethotrexateandcytarabine.Addeddoseandschedule:Age-baseddosing.◊CNSdiseasewithanyhistology:days3,10,and17ofcourses1and2.◊CNS-negativediseasewithlargecelllymphoma:day3ofcourse1only.◊CNS-negativediseasewithB-celllymphomaandB-cellacutelymphoblasticleukemia:day3ofeachcycle.•Footnotefnew:AnFDA-approvedbiosimilarisanappropriatesubstituteforrituximab.PBCL-B(8of9)pTableheadingrevised:Age-BasedDosingforITMethotrexate,Cytarabine,HydrocortisoneforTherapiesOtherthanRICE.pNewtableadded:Age-BasedDosingforITMethotrexate,CytarabineforRICE.PBCL-B(9of9)•ReferencespReference6updated:Minard-ColinV,AuperinA,PillonM,etal.Rituximabforhigh-risk,matureB-cellnon-Hodgkin'slymphomainChildren.NEnglJMed2020;382:2207-2219.PBCL-D(2of5)PrinciplesofSupportiveCare•TumorLysisSyndromepSub-bullet2,6thbulletrevised:Allopurinolandrasburicasearegenerallynotgivenconcurrently.ForongoingcontrolofTLS,considerrestartingallopurinolafterrasburicasetherapyiscompleted.PBCL-D(3of5)•RiskofInfectionpBullet2revised:TheremaybeariskofhepatitisBreactivationduringtreatmentwithrituximab.ScreeningforchronicorresolvedhepatitisBviralinfectionshouldbeperformedbeforestartingtreatmentwithrituximab.IfpatientispositiveforhepatitisB,consultwithinfectiousdiseasespecialistandmonitorforreactivationduringandaftertreatmentwithrituximab.pBullet3revised:TheremaybeariskofhepatitisBreactivationduringtreatmentwithrituximab.Antiviralprophylaxisisrecommended.PrintedbyMinTangon3/14/20227:09:12AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.NCCNGuidelinesVersion3.2021PediatricAggressiveMatureB-CellLymphomasdexPEDIATRICAGGRESSIVEMATUREB-CELLLYMPHOMAaDIAGNOSISettlymphomaandPediatricdiffuselargeB-celllymphoma(DLBCL)includingadolescentandyoungadult(AYA)patientsologysettingbcologysettingbcd•BiopsypExcisionalorincisionalbiopsyofmostaccessiblesiteispreferred.pTouchpreparationsoffreshlesionaltissueshouldbeencouragedwheneverpossiblesince,ifdoneproperly,theymayrevealessentialcytologicdetailsthatmaybedifficulttodetectinsmallbiopsies(eg,smallneedlecorebiopsy).pAcoreneedlebiopsyislessoptimalbutcanbeusedincircumstanceswhenalymphnodeortumormassisnoteasilyaccessibleforexcisionalorincisionalbiopsy.pCoresmustbeofsufficientsizeandnumbertoallowforevaluationofmorphology,tumorarchitecture,andallnecessaryancillarystudies(immunohistochemistry[IHC],flowcytometry,karyotype,andFISHformajortranslocations,asapplicable).pAfine-needleaspiration(FNA)biopsyaloneisnotsuitablefortheinitialdiagnosisofpediatriclymphoma.pPlacefreshspecimeninsaline,notformalin,ensuringviablediagnostictissueforPathologyfthePathologyfAdditionalosticCLaPediatricBurkittlymphomaandDLBCLarecurable,butthetreatmentiscomplex.Itispreferredthattreatmentoccuratcenterswithexpertiseinthemanagementofthesediseases.bRecommendationsforthemanagementofprimarymediastinalB-celllymphoma(PMBL)arenotincludedintheseguidelines.ForPMBLfirst-linetherapyrecommendations,seetheadultNCCNGuidelinesforB-CellLymphomas(BCEL-B,page1of3).cThePediatricAggressiveMatureB-CellLymphomaspanelconsiders“pediatric”toincludeanypatientaged18yearsandyounger,andAYApatientsolderthan18yearsofage,whoaretreatedinapediatriconcologysetting.PracticepatternsvarywithregardstoAYApatientsfromcentertocenterintermsofwhetherAYApatients(definedbytheNationalCancerInstituteas<39yearsofage)withmatureB-celllymphomaaretreatedprimarilybypediatricoradultoncologists.TheseguidelinesareintendedtoapplytoAYApatientswithgoodorganfunctiontreatedinapediatriconcologysetting.AYApatientstreatedinanadultoncologysettingshouldbetreatedaspertheadultNCCNGuidelinesforB-CellLymphomas.dAlsoseetheNCCNGuidelinesforAdolescentandYoungAdult(AYA)Oncology.eDefinitivediagnosismaynotbefeasiblebeforebeginningtreatment.Ifthepatientisverysick,morphologyandflowcytometryaretheminimummethodologiesfromwhichtoyielddiagnosticinformationtobegintreatment.Malignantfluidcytologyandflowcytometrymaysuffice.fSeePrinciplesofPathology(PBCL-A).Note:Allrecommendationsarecategory2Aunlessotherwiseindicated.ClinicalTrials:NCCNbelievesthatthebestmanagementofanypatientwithcancerisinaclinicaltrial.Participationinclinicaltrialsisespeciallyencouraged.Version3.2021,03/10/22©2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.PBCL-1orsinglenucleotidepolymorphismSNParrayforqaberrationSHlPrintedbyMinTangon3/14/20227:09:12AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©orsinglenucleotidepolymorphismSNParrayforqaberrationSHlNCCNGuidelinesVersion3.2021PediatricAggressiveMatureB-CellLymphomasdexADDITIONALDIAGNOSTICTESTINGfESSENTIAL•Adequateimmunophenotypingtoestablishdiagnosisg,h,ipImmunohistochemistrypanel:Ki-67,BCL-2,BCL-6,CD3,CD10,CD20,MUM1pFlowcytometry:Surfacekappa/lambdapp•FISHforBCL-2andBCL-6rearrangementskKaryotypet14)orvariantst(2;8)•FISHforBCL-2andBCL-6rearrangementsk•C-MYCimmunohistochemistry•TdTimmunohistochemistryorflowcytometry•Clonalitytestingbypolymerasechainreaction(PCR)forimmunoglobulingenerearrangementfSeePrinciplesofPathology(PBCL-A).gTypicalimmunophenotypeofBurkittlymphoma:sIg+,CD10+,CD20+,TdT-,Ki-67+(≥95%),BCL2-,BCL6+,simplekaryotypewithMYCrearrangementassoleabnormality.TypicalimmunophenotypeofDLBCL:sIg+,CD20+,TdT-,Ki-67variablyhigh,CD10+/-,BCL6+/-,MUM1+/-,BCL2+/-,variablekaryotypewithC-MYC,BCL6,BCL2,and/orotherIgHrearrangements.hSeeUseofImmunophenotyping/GeneticTestinginDifferentialDiagnosisofMatureB-CellandNK/T-CellNeoplasms(NHODG-A)intheNCCNGuidelinesforB-CellLymphomas.iIfflowcytometryisinitiallyperformed,IHCforselectedmarkers(BCL2andKi-67)cansupplementtheflowresults.jOnformalin-fixed,paraffin-embeddedtissue,MYCrearrangementisbestassessedbyMYCbreakapartprobetocaptureanypartnergene.kDouble-andtriple-hitlymphomasarecurrentlynotwelldescribedorstudiedinthepediatricpopulationbutFISHforBCL-2andBCL-6rearrangementsmaybeconsideredintheAYApopulation.lEBER-ISHismostapplicableinendemicBurkittlymphomaorimmunocompromisedclinicalsettingsforeitherBurkittlymphomaorDLBCL.Note:Allrecommendationsarecategory2Aunlessotherwiseindicated.ClinicalTrials:NCCNbelievesthatthebestmanagementofanypatientwithcancerisinaclinicaltrial.Participationinclinicaltrialsisespeciallyencouraged.PBCL-2Version3.2021,03/10/22©2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.ryFISHforMYCrearrangementandemistryofbonemarrowpPrintedbyMinTangon3/14/20227:09:12AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2022ryFISHforMYCrearrangementandemistryofbonemarrowpNCCNGuidelinesVersion3.2021PediatricAggressiveMatureB-CellLymphomasdexWORKUP•History,includingpersonalandfamilyhistoryofimmunodeficiency•Physicalexam,withattentiontolymphnodes,Waldeyer’sring,liverandspleensize,effusions,ascites,neurologicsigns•Evaluationforsignsorsymptomsofureteralorbowelobstruction•Evaluationforsignsorsymptomsofspinalcordcompressionorcranialneuropathy•Performancestatus(Lansky/Karnofsky)•LabspCBCwithdifferentialpElectrolytes,calcium,phosphorus,bloodureanitrogen(BUN),creatinine,uricacidpLactatedehydrogenase(LDH)pAspartatetransaminase(AST),alaninetransaminase(ALT),bilirubin,albuminpHepatitisBtesting(HBcAb,HBsAb,HBsAg)pConsiderHIVtesting,ifindicatedpConsiderG6PDtestingformalepatientsmpPregnancytestforfemalesofchildbearingage•Bilateralbonemarrowaspirateandbiopsy•LumbarpuncturepCellcount&differentialpCytology,includingtotalnucleatedcellcountandmorphologicreviewofcytospind•ImagingpCross-sectionalscansoftheneck,chest,abdomenandpelvis◊NeckCTwithIVcontrastorMRIwithandwithoutcontrast◊ChestCTwithIVcontrast◊AbdomenandpelvisCTwithoralandIVcontrastorMRIwithandwithoutcontrastpFDG-PET/CTorFDG-PET/MRI,ifavailable(donotdelaytreatmenttoobtain)npChestx-rayposteroanterior(PA)/lateralandabdominalultrasound(ifcross-sectionalimagingnotavailable)•Echocardiogram(ECHO)ormultigatedacquisition(MUGA)scanandECG•Fertilitycounselingrecommended;fertilitypreservationasclinicallyappropriateSeeNCCNGuidelinesforAdolescentandYoungAdult(AYA)Oncology•MRIofthehead,ifclinicallyindicatedFlowcytometryofcerebrospinalfluid(CSF)o•MRIFlowcytometryofcerebrospinalfluid(CSF)oRisk GroupDefinitions(PBCL-4)icrludetheneedforfulldiagnosticqualityhigh-resolutionCTorMRI.pForlow-levelormorphologicallyindeterminateinvolvement.oFlowcytometryofCSFsamplesisnotroutinelyrecommended,butmaypForlow-levelormorphologicallyindeterminateinvolvement.Note:Allrecommendationsarecategory2Aunlessotherwiseindicated.ClinicalTrials:NCCNbelievesthatthebestmanagementofanypatientwithcancerisinaclinicaltrial.Participationinclinicaltrialsisespeciallyencouraged.PBCL-3Version3.2021,03/10/22©2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.PrintedbyMinTangon3/14/20227:09:12AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.NCCNGuidelinesVersion3.2021PediatricAggressiveMatureB-CellLymphomasNSqGroupClassificationrCompletelyresectedstageIorCompletelyresectedabdominalstageIICLGroupBAllcasesnoteligibleforGroupAorGroupC(unresectedstageIandnon-abdominalstageII,stageIII,andnon-CNSstageIVwith<25%bonemarrowinvolvement)CLandPBCL-7GroupCAnyCNSinvolvementsand/orBonemarrowinvolvement(≥25%lymphomacells)CLNote:Allrecommendationsarecategory2Aunlessotherwiseindicated.ClinicalTrials:NCCNbelievesthatthebestmanagementofanypatientwithcancerisinaclinicaltrial.Participationinclinicaltrialsisespeciallyencouraged.PBCL-4eLoussssQsssdgougICobLepeueCguceLdeMoLkdCCdVIILapLeeLeqdCCdCnqeIueguqpIIn在Lg在!ou山g入uo在peLebLoqnceq!ugu入LoL山M!在pon在在peexbLe22ML!在在eubeL山!22!ouoLdCCd.<CRzorRefractoryDisease(SeePBCL-10)PrintedbyMinTangon3/14/20227:09:12AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2022NationalComprehensiveCancerNetwork,Inc.,<CRzorRefractoryDisease(SeePBCL-10)NCCNGuidelinesVersi