软组织肉瘤2022.v1（英文）-NCCN肿瘤临床实践指南

NCCNClinicalPracticeGuidelinesinOncologyNCCNGuidelines®)SoftTissueSarcomaersionMarchNCCNGuidelinesforPatients®availableat/patientsVersion1.2022,03/29/22©2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.PrintedbyMinTangon4/1/202211:18:51AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.a*MargaretvonMehren,MD/Chair†FoxChaseCancerCenter*JohnM.Kane,III,MD/Vice-Chair¶CancerCenterMarkAgulnik,MD†CityofHopeNationalMedicalCenterMarilynM.Bui,MD,PhD≠MoffittCancerCenterJanaiCarr-Ascher,MD,PhD≠†UCDavisComprehensiveCancerCenterEdwinChoy,MD,PhD†CancerCenterSarahDry,MD≠UCLAJonssonComprehensiveCancerCenterKristenN.Ganjoo,MD†RicardoJ.Gonzalez,MD¶MoffittCancerCenterAshleyHolder,MD¶CancerCenteratUABJadeHomsi,MD†esPanelDisclosuresVickiKeedy,MD,MSCI†Vanderbilt-IngramCancerCenterCiaraM.Kelly,MD†EdwardKim,MD§SeattleCancerCareAllianceDavidLiebner,MDÞ†CancerCenterJamesCancerHospitalMartinMcCarter,MD¶UniversityofColoradoCancerCenterSeanV.McGarry,MD¶τChristianMeyer,MD,PhD†kinsAlbertoS.Pappo,MD€AmandaM.Parkes,MD‡†UniversityofWisconsinIvyA.Petersen,MD§SethM.Pollack,MD†MatthewPoppe,MD§RichardF.Riedel,MD†ScottSchuetze,MD,PhD†ofMichiganRogelCancerCenterJacobShabason,MD§nterJasonK.Sicklick,MD¶cerCenterMatthewB.Spraker,MD,PhD§SitemanCancerCenteratBarnes-JewishHospitalandWashingtonUniversitySchoolofMedicineMelissaZimel,MDτ¶UCSFHelenDillerFamilyCenterangPhD‡Hematology/oncologyÞInternalmedicine†Medicaloncology§Radiotherapy/Radiationoncology¶Surgery/SurgicalPrintedbyMinTangon4/1/202211:18:51AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.adexlievesthatthebestmanagementlievesthatthebestmanagementforanypatientwithcancerisinaclinicaltrial.Participationinclinicaltrialsisespeciallyencouraged.FindanNCCNMemberInstitution:/home/member-institutions.ofEvidenceanddationsotherwisedNCategoriesofEvidenceandConsensus.NCCNCategoriesofPreference:Allrecommendationsareconsideredappropriate.SeeNCCNCategoriesofPreference.SummaryoftheGuidelinesUpdatesSoftTissueSarcoma•Extremity/BodyWall,Head/Neck(EXTSARC-1)•Retroperitoneal/Intra-Abdominal(RETSARC-1)•DesmoidTumors(AggressiveFibromatosis)(DESM-1)•Rhabdomyosarcoma(RMS-1)PrinciplesofImaging(SARC-A)PrinciplesofPathologicAssessmentofSarcomaSpecimens(SARC-B)PrinciplesofAncillaryTechniquesUsefulintheDiagnosisofSarcomas(SARC-C)PrinciplesofSurgery(SARC-D)PrinciplesofRadiationTherapy(SARC-E)SystemicTherapyAgentsandRegimenswithActivityinSoftTissueSarcomaSubtypes(SARC-F)PrinciplesofCancerRiskAssessmentandCounseling(SARC-G)ficationSTBoneSarcomas-SeetheNCCNGuidelinesforBoneCancerGastrointestinalStromalTumors-SeetheNCCNGuidelinesforGastrointestinalStromalTumorsUterineSarcomas-SeetheNCCNGuidelinesforUterineNeoplasmsDermatofibrosarcomaProtuberanswithoutFibrosarcomatousTransformation-SeetheNCCNGuidelinesforDermatofibrosarcomaProtuberansTheNCCNGuidelinesareastatementofevidenceandconsensusoftheauthorsregardingtheirviewsofcurrentlyacceptedapproachestotreatmentAnyclinicianseekingtoapplyorconsulttheNCCNGuidelinesisexpectedtouseindependentmedicaljudgmentinthecontextofindividualstancestodetermineanypatientscareortreatmentTheNationalComprehensiveCancerNetworkNCCNmakesnorepresentationsorwarrantiesofanykindregardingtheircontentuseorapplicationanddisclaimsanyresponsibilityfortheirapplicationoruseinanywayTheNCCNbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.©2022.Version1.2022,03/29/22©2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.UPDATESPrintedbyMinTangon4/1/202211:18:51AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.UPDATESadexUpdatesinVersion1.2022oftheNCCNGuidelinesforSoftTissueSarcomafromVersion3.2021include:Globalchange:"preoperative"changedto"neoadjuvant"and"postoperative"to"adjuvant"EXTSARC-1•Workup,EssentialpBullet5,"Chestimaging"deleted;modifiedasfollows:ImagingofpotentialsitesofmetastaticdiseasepBullet7,sub-bullet3modifiedasfollows:Forhereditarynon-polyposiscolorectalcancer(HNPCCorLynchsyndrome),SeeNCCNGuidelinesforGenetic/ •Specialconsiderationsforuniquehistologies,newtextaddedunder"Rhabodomyosarcoma(RMS)":Dermatofibrosarcomaprotuberans(DFSP)withoutfibrosarcomatoustransformation.tnotesp"b"modifiedMRIwithandwithoutcontrast±"and/or"CTwithcontrast.p"c"deleted:Inselectedinstitutionswithclinicalandpathologicexpertise,afine-needleaspirationbiopsy(FNAB)maybeacceptable.p"h"modifiedtoinclude:forothersofttissuesarcomasoftheextremity/bodywall,head/neck(EXTSARC-1andEXTSARC-5).SeeSARC-F,2of11.EXTSARC-2tnotespCombinedfootnotes"m"and"n":NeoadjuvantRTispreferredinthesetherareselectedcircumstances(eg,wideresectiontoobtainnegativemarginswouldbetechnicallychallengingorresultinsignificantmorbidityorpriortore-resectionfollowingR2resection).pmInthesettingwherewidesurgicalmarginsmaybedifficultormorbid,neoadjuvantradiationmaybeanoption.pnItmaybeappropriatetoconsiderRTpriortore-resectionforR2resections.p"m"modified:Treatmentoptionsincludingre-resectionrevisionsurgeryEXTSARC-3•Follow-UppBullet4modified:Obtainend-of-treatmentadjuvantbaselinetnotespFootnote"r"isnew:Formanagementofaprimarysarcomawithsynchronousregionalnodalmetastaticdisease,seeabovefortreatmentoftheprimarytumorandrefertoEXTSARC-6formanagementofnodaldisease.(AlsoforEXTSARC-4)pThetextforfootnote"s"wasmovedtoSARC-EandthelinkremainsdirectingthereadertothePrinciplesofRadiationTherapy.EXTSARC-5•PrimaryTreatmentpBullet3modified:Forlungmetastases,resection(preferred)orstereotacticbodyradiationtherapy(SBRT)combinedtextfrombullet4.pMetastasesaddedto"embolizationprocedures(non-lung)"forupperandlowerpathways.(AlsoforEXTSARC-6)tnotesp"ee"modifiedtoinclude:BaumannBC,etal.JSurgOncol2020;122:877-883.(AlsoforEXTSARC-6)p"gg"deleted:PalliativeRTrequiresbalancingexpedienttreatmentwithsufficientdoseexpectedtohaltthegrowthoforcausetumorregression.Numerousclinicalissuesregardingrapidityofgrowth,thestatusofsystemicdisease,andtheuseofsystemictherapymustbeconsidered.RecommendedonlyforpalliativetherapyinpatientswithsynchronousstageIVorrecurrentdiseasewithdisseminatedmetastases.(Alsofor•IsolatedregionaldiseaseornodespDeletedthefollowingunderoptions:◊Metastasectomy±neoadjuvantoradjuvantsystemictherapy±RT◊SBRT◊Isolatedlimbperfusion/infusion±surgeryVersion1.2022,03/29/22©2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.UPDATESVersion1.2022,03/29/22©2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.PrintedbyMinTangon4/1/202211:18:51AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.adexUpdatesinVersion1.2022oftheNCCNGuidelinesforSoftTissueSarcomafromVersion3.2021include:(continued)p"aa"deleted:Shouldonlybedoneatinstitutionswithexperienceinisolatedlimbperfusion/infusion.RETSARC-1•WorkuppBullet4modified:Image-guidedcoreneedlebiopsyshouldbeperformedifneoadjuvanttherapyisbeingconsideredgivenorforsuspicionofmalignancyotherthansarcoma.pBullet5modified:Preresectionbiopsyisnotnecessarilyrequired.forwell-differentiatedliposarcoma.RETSARC-2•PrimaryTreatmentpSarcoma,Neoadjuvanttherapy:(inselectedcases)added.(AlsoforRETSARC-5)•PrimaryTreatmentFirstsub-bullet:(ifnotpreviouslygivenfortheprimarytumor)addedtoRT(AlsoforRETSARC-5)tnotesp"j"modified:Considerpostpreoperativesystemictherapyforhistologieswithifhighriskformetastaticdiseaseand/orhighriskforlocalrecurrence.Systemictherapyisnotrecommendedforlow-gradetumors.(AlsoforRETSARC-3,RETSARC-5)RETSARC-3•SurgicalOutcomespRO:ConsideradjuvantsystemictherapyforhistologieswithifhighriskformetastaticdiseasepRecommendationsforR1andR2wereseparatedintodifferentbranches.pR1:AdjuvantRTshouldnotbeadministeredroutinelywiththeexceptionofhighlyselectedpatientsandunlesslocalrecurrencewouldcauseunduemorbidity(AlsoforR2)pR2:Inhighlyselectedcases,considerboost(10-16Gy)ifneoadjuvantRTwasgiven.•Bullet1modified:Observation,ifasymptomaticandindolenttumorbiologyRETSARC-5AddedConsiderbeforeneoadjuvanttherapy.tnotesp"t"modified:Consideradjuvantsystemictherapyforhistologieswithifhighriskformetastaticdiseaseorhistoryofseveralrecurrenceswithahighriskforadditionallocalrecurrences.p"u"deleted:IfnopriorRTforthetreatmentoftheprimarysarcoma.DESM-1•WorkuppBullet3modified:ConsiderevaluationforGardner'ssyndrome/familialadenomatouspolyposis(FAP)ifbiopsyisdiagnosticofdesmoidpBullet4modified:AppropriateimagingofprimarysitewithCTorMRIasclinicallyindicatedtnotesp"b"deleted:SeePrinciplesofImaging(SARC-A)(AlsoforDESM-2,DESM-3).DESM-2•Column2:ObservationwithimagingwithCTorMRIasindicatedandsymptommanagement(AlsoforDESM-3)ColumnpStable/regression:ContinueobservationwithimagingwithCTorMRIasindicated(AlsoforDESM-3)pProgression:ConsiderongoingobservationwithimagingwithCTorMRIasindicated•Footnote"d,"secondsentencemodifiedtoinclude"initial"imagingevery...(AlsoforDESM-3).UPDATESVersion1.2022,03/29/22©2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.PrintedbyMinTangon4/1/202211:18:51AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.adexnoftheNCCNGuidelinesforSoftTissueSarcomafromVersioninclude•Titlechanged:TreatmentBasedonAnatomicLocation.ActiveTherapyforProgressive,Morbid,orSymptomaticDisease•Significantlymodifiedthepage.SARC-A•PrinciplesofImagingpNewtableincorporatestextfrompreviouspages.SARC-C(1of3)•Newstatementaddedtopage:Next-generationsequencing(NGS),includingDNAandRNAsequencing,maybebeneficialinselectedpatients.ThetimingofwhentoperformNGSandforwhichpatientsmustbeevaluatedindividually.NGSfindingscanhelppatientsqualifyforclinicaltrialsandcanidentifyactionablemutationsthatmaynothavebeentargetedbypriortherapies.Thus,NGSmaybeappropriateforpatientswhomayqualifyforandwhoareinterestedinenrollinginaclinicaltrialorforpatientswithdiseasethatisrefractorywhohavefailedorprogressedonstandardtherapiesorincertainhistologieswhereNGSprovidesclinicallyactionableinformation.NGSshouldnotreplaceexpertpathologyreview,asNGSonlyrarelyresultsinadiagnosischangefollowingexpertreview.TechnicallysuccessfulNGSonbonebiopsiesrequiresuseofdecalcificationagents,suchasEDTA,thatdonotinterferewithgenomictesting.SARC-C(3of3)•Thefollowinggenesarenewforinflammatorymyofibroblastictumor:ETV6-NTRK3andTFG-ROS1•Thefollowingreferencesarenew:pTaylorMS,ChouguleA,MacLeayAR,etal.MorphologicoverlapbetweeninflammatorymyofibroblastictumorandIgG4-relateddisease:Lessonsfromnext-generationsequencing.AmJSurgPathol2019;43:314-324.pLopez-NunezO,JohnI,PanasitiRN,etal.Infantileinflammatorymyofibroblastictumors:clinicopathologicalandmolecularcharacterizationof12cases.ModPathol2020;33:576-590.pLovlyCM,GuptaA,LipsonD,etal.Inflammatorymyofibroblastictumorsharbormultiplepotentiallyactionablekinasefusions.CancerDiscov9-895.•PrinciplesofSurgery◊Firstbulletmodified:Apreoperativeneoadjuvantpathologicdiagnosis,includinghistologicsubtypeandgrade,isalmostalwaysnecessaryfortheoptimaltreatmentofasofttissuesarcoma(surgicalresectionmarginplanning,adiscussionofneoadjuvantchemotherapy,and/orradiation)◊Secondbulletmodified:Percutaneouscoreneedlebiopsyispreferredasitisassociatedwithalowriskforbiopsy-relatedcomplications.Thebiopsytractshouldavoidpotentialtumorcontaminationofuninvolvedanatomiccompartmentsand,ideally,beinlinewithanyfuturesurgicalresectionincision.Incertainsituations,especiallydeep-seatedtumors,image-guidedneedlebiopsycanimprovediagnosticaccuracy(avoidnecroticnondiagnosticareasorsurroundingnormaltissues,andthoroughlysampleheterogenoustumors).Openincisionalbiopsycanbeconsideredifpercutaneouscoreneedlebiopsiesfailtoleadtomakeanadequatediagnosis.Apretreatmentbiopsytodiagnoseandgradeasarcomaishighlypreferred.Biopsyshouldbecarriedoutbyanexperiencedsurgeon(orradiologist)andmaybeaccomplishedbyopenincisionalorneedletechnique.Coreneedlebiopsyispreferred;however,anopenincisionalbiopsymaybeconsideredbyanexperiencedsurgeon.Image-guidedneedlebiopsymaybeindicatedforextremity/truncalsarcomas.pSurgery◊Bullet1modified:Thesurgicalprocedurenecessarytoresectthetumorwithoncologicallyappropriatemarginsshouldbeused.Ideally,thiswouldbepathologicallynegativeresectionmargins.However,plannedclosemarginsorevenmicroscopicallypositivemarginsmaybenecessaryappropriatetopreservecriticalneurovascularstructures(eg,majorvessels,nerves,bones,joints),especiallyinthesettingofmultimodalitytherapy.◊Bullet2modified:Evaluateneoadjuvantlyforrehabilitationpriortosurgery(seeSARC-D2of2).PrintedbyMinTangon4/1/202211:18:51AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.adexrsionoftheNCCNGuidelinesforSoftTissueSarcomafromVersionincludeSARC-E(1through4)•PrinciplesofRadiationTherapyforSoftTissueSarcomapThissectionoftheguidelineshasbeensignificantlymodified.SARC-F(1of11)•SystemicTherapyAgentsandRegimenswithActivityinSoftTissueSarcomapFootnotescorrespondingtothetitle:◊"c"modified:Includingbutnotlimitedtoalveolarsoftpartsarcoma(ASPS),ALT/WDLS,andclearcellsarcomas,whicharegenerallynotsensitive.tocytotoxicsystemictherapy◊"d"isnew:Dexrazoxanemaybeaddedasacardioprotectantforthepreventionofcardiotoxicityinpatientsplanningtoreceivehigh-doseanthracyclines(eg,doxorubicin>250mg/m2).ArmenianSH,etal.JClinOncol2017;35:893-911.pPreferred,First-lineTherapyAdvanced/Metastatic◊NTRKgenefusion-positivesarcomasonly(movedfromUsefulinCertainCircumstancescolumn)–Larotrectinib–Entrectinib•UsefulinCertainCircumstancespFirst-lineTherapyAdvanced/Metastatic◊Pazopanib(patientsineligibleforIVsystemictherapyorpatientswhoarenotcandidatesforanthracycline-basedregimens)•OtherRecommendedRegimenspNeoadjuvant/AdjuvantTherapy◊Bullet1:ADLMSonly(doxorubicin,dacarbazine)-ififosfamideisnotconsideredappropriate•OtherRecommendedRegimenspSubsequentLinesofTherapyforAdvanced/MetastaticDisease◊Bullet6:Gemcitabine-basedregimens(ifnotgivenpreviously)◊Sub-bullet5:anewregimen,Gemcitabineandpazopanibisacategory2Brecommendation•UsefulinCertainCircumstancespSubsequentLinesofTherapyforAdvanced/MetastaticDisease◊Pembrolizumab–Footnote"k":Forthetreatmentofpatientswithunresectableormetastatictumormutationalburden-high(TMB-H)[≥10mutations/megabase(mut/Mb)]tumors,asdeterminedbyanFDA-approvedtest,thathaveprogressedfollowingpriortreatmentandwhohavenosatisfactoryalternativetreatmentoptions.SARC-F(2of11)•DesmoidTumors(AggressiveFibromatosis)pDeleted:Timetoresponse"less"and"more"critical.pFootnote"l,"OptimaldurationofTKItherapyhasnotbeenestablished.DiscontinuationofTKItherapycanbeconsidered(withcarefulmonitoring)inpatientswithstabledisease,isnewcorrespondingtothetitle.•Non-PleomorphicRhabdomyosarcoma◊"m":RemovedfromtheheaderandplacednexttoallinstancesofVACandVAI.•OtherRecommendedRegimenspVinorelbine/cyclophosphamide/temsirolimusaddedasanewregimen.Version1.2022,03/29/22©2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.UPDATESPrintedbyMinTangon4/1/202211:18:51AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.adexrsionoftheNCCNGuidelinesforSoftTissueSarcomafromVersionincludeSARC-F(2of11)(continued)◊Newreference:MascarenhasL,ChiYY,HingoraniP,etal.RandomizedphaseIItrialofbevacizumabortemsirolimusincombinationwithchemotherapyforfirstrelapserhabdomyosarcoma:areportfromtheChildren'sOncologyGroup.JClinOncol2019;37:2866-2874.•UsefulinCertainCircumstancespMaintenancechemotherapy(cyclophosphamide/vinorelbine)forpatientswithintermediate-riskRMSwithCRfollowingtreatmentwithVACorVAIregimen(pleasenote:COGhasanactiveprospectiveongoingstudy,butconsideredareasonablestandardofcare).◊Newreference:BisognoG,DeSalvoGL,BergeronC,etal.Vinorelbineandcontinuouslow-dosecyclophosphamideasmaintenancechemotherapyinpatientswithhigh-riskrhabdomyosarcoma(RMS2005):amulticenter,open-label,randomised,phase3trial.LancetOncol2019;20:1566-1575.SARC-F(3of11)•AlveolarSoftPartSarcoma(ASPS)pPreferredRegimens◊Pembrolizumabincombinationwithaxitinibaddedasanewregimen◊Newreference:WilkyBA,TruccoMM,SubhawongTK,etal.Axitinibpluspembrolizumabinpatientswithadvancedsarcomasincludingalveolarsoft-partsarcoma:asingle-centre,singlearm,phase2trial.LancetOncol2019;20:837-848.sarcomapOtherRecommendedRegimens◊Movedsorafenib,sunitinib,andbevacizumabtoUsefulincertaincircumstances◊Deleted:AllothersystemictherapyoptionsrecommendedforSoftTissueSarcomaSubtypeswithNon-SpecificHistologiespUsefulinCertainCircumstances◊Regorafenibaddedasanewregimenwiththefollowingreference:AgulnikM,SchulteB,RobinsonS,etal.Anopen-labelsingle-armphaseIIstudyofregorafenibforthetreatmentofangiosarcoma.EurJCancer2021;154:201-208.◊Pembrolizumab(forcutaneousangiosarcoma)addedasanewregimenwiththefollowingreference:FlorouV,RosenbergAE,WiederE,etal.Angiosarcomapatientstreatedwithimmunecheckpointinhibitors:acaseseriesofsevenpatientsfromasingleinstitution.JImmunotherCancer2019;7:285.SARC-F(4of11)•DermatofibrosarcomaProtuberans(DFSP)withFibrosarcomatousTransformationpPreferredRegimens◊Imatinibaddedasanewregimenwiththefollowingreference:RutkowskiP,KlimczakA,LugowskiI,etal.Long-termresultsoftreatmentofadvanceddermatofibrosarcomaprotuberans(DFSP)withimatinibmesylate-Theimpactoffibrosarcomatoustransformation.EurJSurgOncol2017;43:1134-41.Version1.2022,03/29/22©2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.UPDATESUPDATESVersion1.2022,03/29/22©2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.PrintedbyMinTangon4/1/202211:18:51AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.adexionoftheNCCNGuidelinesforSoftTissueSarcomafromVersionincludeSARC-F(4of11)(continued)•DermatofibrosarcomaProtuberans(DFSP)withFibrosarcomatousTransformationpOtherRecommendedRegimens◊AllothersystemictherapyoptionsrecommendedforSoftTissueSarcomaSubtypeswithNon-SpecificHistologies–Anthracycline-basedregimens:▪Doxorubicin▪Epirubicin▪Liposomaldoxorubicin▪AIM(doxorubicin,ifosfamide,mesna)▪Ifosfamide,epirubicin,mesna▪MAID(mesna,doxorubicin,ifosfamide,dacarbazine)–Gemcitabine-basedregimens:▪Gemcitabine▪Gemcitabineanddocetaxel▪Gemcitabineandvinorelbine▪Gemcitabineanddacarbazine–Pazopanib(patientsineligibleforIVsystemictherapyorpatientswhoarenotcandidatesforanthracycline-basedregimens)SARC-F(5of11)•InflammatoryMyofibroblasticTumor(IMT)withAnaplasticLymphomaKinase(ALK)TranslocationpPreferredRegimens◊Lorlatinibaddedasanewregimen.UPDATESVersion1.2022,03/29/22©2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.PrintedbyMinTangon4/1/202211:18:51AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.adexrsionoftheNCCNGuidelinesforSoftTissueSarcomafromVersionincludeSARC-F(6of11)•SolitaryFibrousTumorpOtherRecommendedRegimens◊AllothersystemictherapyoptionsrecommendedforSoftTissueSarcomaSubtypeswithNon-SpecificHistologies◊Anthracycline-basedregimens:–Doxorubicin–Epirubicin–Liposomaldoxorubicin–AD(doxorubicin,dacarbazine)–AIM(doxorubicin,ifosfamide,mesna)–Ifosfamide,epirubicin,mesna–MAID(mesna,doxorubicin,ifosfamide,dacarbazine)◊Gemcitabine-basedregimens:–Gemcitabine–Gemcitabineanddocetaxel–Gemcitabineandvinorelbine–Gemcitabineanddacarbazine◊Trabectedin•TenosynovialGiantCellTumor/PigmentedVillonodularSynovitispUsefulinCertainCircumstances◊Nilotinibaddedasanewregimenwiththefollowingreference:GelderblomH,CropetC,ChevreauC,et.al.Nilotinibinlocallyadvancedpigmentedvillonodularsynovitis:amulticentre,open-label,single-arm,phase2trial.LancetOncol2018;19:639-648.•PrinciplesofCancerRiskAssessmentandCounselingpThisisanewpagediscussingwhentoconsidergenetictestingforinheritedsofttissuesarcomas.PrintedbyMinTangon4/1/202211:18:51AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.dexWORKUP•Priortotheinitiationoftherapy,itishighlyrecommendedthatallpatientsbeevaluatedandmanagedbyamultidisciplinaryteamwi