软组织肉瘤2022.v2-NCCN（英文版）

NCCNClinicalPracticeGuidelinesinOncologyNCCNGuidelines®)SoftTissueSarcomaersionMayNCCNGuidelinesforPatients®availableat/patientsVersion2.2022,05/17/22©2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.PrintedbyMinTangon6/4/20227:43:38AM.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/SurgicalPrintedbyMinTangon6/4/20227:43:38AM.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.Version2.2022,05/17/22©2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.PrintedbyMinTangon6/4/20227:43:38AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.adexnoftheNCCNGuidelinesforSoftTissueSarcomafromVersioninclude•RTmodified:(considerfortumorsathighriskforlocalrecurrence)(ifnotpreviouslygivenfortheprimarytumor).•Footnote"j",modified:Considersystemictherapyifhighriskformetastaticdiseaseorifdownstagingisneededtofacilitateresection.and/orhighriskforlocalrecurrence.Systemictherapyisnotrecommendedforlow-gradetumors.(AlsoforRETSARC-3andRETSARC-5).SARC-E(3of4)NeoadjuvantRT,modified:•NeoadjuvantRTforretroperitoneal/intra-abdominalsarcomascanbeconsideredinselectedpatientsathighriskforlocalrecurrence.•IfneoadjuvantRTisdeemedtobeappropriateforapatient,thefollowingGeneraldoseguidelinesarerecommendedpThefollowingreferenceisnew:BonvalotS,GronchiA,LePéchouxC,etal.Preoperativeradiotherapyplussurgeryversussurgeryaloneforpatientswithprimaryretroperitonealsarcoma(EORTC-62092:STRASS):amulticentre,open-label,randomised,phase3trialLancetOncol2020;21:1366-1377.MS-1•SectionsoftheDiscussionhavebeenupdatedtoreflectthechangesinthealgorithm.UpdatesinVersion1.2022oftheNCCNGuidelinesforSoftTissueSarcomafromVersion3.2021include:Globalchange:Globalchange:"preoperative"changedto"neoadjuvant"and"postoperative"to"adjuvant"EXTSARC-1pEssentialp""postoperative"to"adjuvant"EXTSARC-1pEssentialEXTSARC-3•Follow-UpdasfollowsImagingdasfollowsImagingofpotentialsitesofpotentialsitesofmetastaticdiseaseorectalcancerorectalcancerHNPCCorLynchsyndromeSeeNCCNGuidelinesforcFamilialHighRiskAssessmentBreastOvarianandPancreaticdelinesforGeneticFamilialHighRiskAssessmentColorectalsynchronousregionalnodalmetastaticdisease,seeabovefortreatmentoftheprimarytumorandrefertoEXTSARC-6formanagementofnodaldisease.(AlsoforEXTSARC-4)AlsoforRETSARC)pThetextforfootnote"s"wasmovedtoSARC-EAlsoforRETSARC)Specialconsiderationsforuniquehistologies,newtextaddedunderSpecialconsiderationsforuniquehistologies,newtextaddedunder"Rhabodomyosarcoma(RMS)":Dermatofibrosarcomaprotuberans(DFSP)withoutfibrosarcomatoustransformation.tnotesithcontrastEXTSARC-5•PrimaryTreatmentpBullet3modified:Forlungmetastases,resection(preferred)orstereotacticbodyradiationtherapy(SBRT)combinedtextfrombullet4.tiseafine-needleaspirationbiopsy(FNAB)maytiseafine-needleaspirationbiopsy(FNAB)maybeacceptable.suesarcomasoftheextremitybodywall,head/neck(EXTSARC-1andEXTSARC-5).SeeSARC-F,2of11.tnotesbodywall,head/neck(EXTSARC-1andEXTSARC-5).SeeSARC-F,2of11.tnotestherareselectedcircumstances(eg,wideresectiontoobtainnegativemarginswouldbetechnicallychallengingorresultinsignificantmorbidityorpriortore-resectionfollowingR2resection).p"gg"deleted:PalliativeRTrequiresbalancingexpedienttreatmentwithsufficientdoseexpectedtohaltthegrowthoforcausetumorregression.Numerousclinicalissuesregardingrapidityofgrowth,thestatusofsystemicdisease,andtheuseofsystemictherapymustbeconsidered.RecommendedonlyforpalliativetherapyinpatientspnItmaybeappropriatetoconsiderRTpriorpnItmaybeappropriatetoconsiderRTpriortoreresectionforRContinuedVersion2.2022,05/17/22©2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.UPDATESUPDATESVersion2.2022,05/17/22©2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.PrintedbyMinTangon6/4/20227:43:38AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.adexnoftheNCCNGuidelinesforSoftTissueSarcomafromVersioninclude•IsolatedregionaldiseaseornodespDeletedthefollowingunderoptions:◊Metastasectomy±neoadjuvantoradjuvantsystemictherapy±RT◊SBRTIsolatedlimbperfusion/infusion±surgerytnotesp"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,ifasymptomaticandindolenttumorbiologyAddedConsiderbeforeneoadjuvanttherapy.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).•Footnote"d,"secondsentencemodifiedtoinclude"initial"imagingevery...(AlsoforDESM-3).DESM-2•Column2:ObservationwithimagingwithCTorMRIasindicatedandsymptommanagement(AlsoforDESM-3)•Column4:pStable/regression:ContinueobservationwithimagingwithCTorMRIasindicated(AlsoforDESM-3)pProgression:ConsiderongoingobservationwithimagingwithCTorMRIasindicatedDESM-4•Titlechanged:TreatmentBasedonAnatomicLocation.ActiveTherapyforProgressive,Morbid,orSymptomaticDisease•Significantlymodifiedthepage.SARC-A•PrinciplesofImagingpNewtableincorporatestextfrompreviouspages.PrintedbyMinTangon6/4/20227:43:38AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.adexrsionoftheNCCNGuidelinesforSoftTissueSarcomafromVersionincludeSARC-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,idesandldbecarriedoutbyanexperiencedsurgeon(orradiologist)andmaybeaccomplishedbyopenincisionalorneedletechnique.Coreneedlebiopsyispreferred;everanopenincisionalbiopsymaybeconsideredbyanexperiencedsurgeonImage-guidedneedlebiopsymaybeindicatedforextremity/truncalsarcomas.pSurgery◊Bullet1modified:Thesurgicalprocedurenecessarytoresectthetumorwithoncologicallyappropriatemarginsshouldbeused.Ideally,thiswouldbepathologicallynegativeresectionmargins.However,plannedclosemarginsorevenmicroscopicallypositivemarginsmaybenecessaryappropriatetopreservecriticalneurovascularstructures(eg,majorvessels,nerves,bones,joints),especiallyinthesettingofmultimodalitytherapy.◊Bullet2modified:Evaluateneoadjuvantlyforrehabilitationpriortosurgery(seeSARC-D2of2).Version2.2022,05/17/22©2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.UPDATES◊Bullet1:ADLMSonly(doxorubicin,dacarbazine)-ififosfamideisnotconsideredappropriate•OtherRecommendedRegimenssefulinCertain◊Bullet1:ADLMSonly(doxorubicin,dacarbazine)-ififosfamideisnotconsideredappropriate•OtherRecommendedRegimenssefulinCertainCircumstances◊Pembrolizumab–Footnote"k":Forthetreatmentofpatientswithunresectableormetastatictumormutationalburden-high(TMB-H)[≥10mutations/megabase(mut/Mb)]tumors,asdeterminedbyanFDA-approvedtest,thathaveprogressedfollowingpriortreatmentandwhohavenosatisfactoryalternativetreatmentoptions.adexUpdatesinVersion1.2022oftheNCCNGuidelinesforSoftTissueSarcomafromVersion3.2021include:SARCEthrough4)SARC-FSARCEthrough4)rinciplesofRadiationTherapyforrinciplesofRadiationTherapyforSoftTissueSarcomapDeleted:f•SystemicTherapyAgentsandRegimenswithActivityinSoftTissuecomapFootnote"l,"OptimaldurationofTKItherapyhasnotbeenestablished.DiscontinuationofTKIf•SystemicTherapyAgentsandRegimenswithActivityinSoftTissuecoma◊"c"modified:◊"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/MetastaticpFootnotes◊"m":RemovedfromtheheaderandplacednexttoallinstancesofVACandVAI.•OtherRecommendedRegimenspVinorelbine/cyclophosphamide/temsirolimusaddedasanewregimen.SARC-F(2of11)(continued)ivesarcomasonlymovedfromUsefulinnCircumstancescolumn–Larotrectinib–EntrectinibsefulinCertainCircumstances◊Newreference:MascarenhasL,ChiYY,HingoraniP,etal.RandomizedphaseIItrialofbevacizumabivesarcomasonlymovedfromUsefulinnCircumstancescolumn–Larotrectinib–EntrectinibsefulinCertainCircumstances•UsefulinCertainCircumstances◊Pazopanib(patientsineligibleforIVsystemictherapyorpatientswhoarenotcandidatesforanthracycline-basedregimens)•OtherRecommendedRegimenspMaintenancechemotherapy◊Pazopanib(patientsineligibleforIVsystemictherapyorpatientswhoarenotcandidatesforanthracycline-basedregimens)•OtherRecommendedRegimensconsideredareasonablestandardofcare).◊Newreference:BisognoG,DeSalvoGL,BergeronC,etal.Vinorelbineandcontinuouslow-dosecyclophosphamideasmaintenancechemotherapyinpatientswithhigh-riskrhabdomyosarcoma(RMS2005):amulticenter,open-label,randomised,phase3trial.LancetOncol2019;20:1566-1575.◊Bullet6:Gemcitabine-basedregimens(if◊Bullet6:Gemcitabine-basedregimens(ifnotgivenpreviously)◊Sub-bullet5:anewregimen,Gemcitabineandpazopanibisacategory2Brecommendation•AlveolarSoftPartSarcoma(ASPS)pPreferredRegimens◊Pembrolizumabincombinationwithaxitinibaddedasanewregimen◊Newreference:WilkyBA,TruccoMM,SubhawongTK,etal.Axitinibpluspembrolizumabinpatientswithadvancedsarcomasincludingalveolarsoft-partsarcoma:asingle-centre,singlearm,phase2trial.LancetOncol2019;20:837-848.sarcomapOtherRecommendedRegimens◊Movedsorafenib,sunitinib,andbevacizumabtoUsefulincertaincircumstancesVersion2.2022,05/17/22©2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.UPDATESUPDATESVersion2.2022,05/17/22©2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.PrintedbyMinTangon6/4/20227:43:38AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.adexrsionoftheNCCNGuidelinesforSoftTissueSarcomafromVersionincludeSARC-F(3of11)(continued)◊Deleted:AllothersystemictherapyoptionsrecommendedforSoftDeleted: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;43:1134-1141.SARC-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.SARC-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.PrintedbyMinTangon6/4/20227:43:38AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.dexWORKUP•Priortotheinitiationoftherapy,itishighlyrecommendedthatallpatientsbeevaluatedandmanagedbyamultidisciplinaryteamwithexpertiseandexperienceinsarcomaa•H&P•Adequateimagingofprimarytumorbisindicatedforalllesionswithareasonablechanceofbeingmalignant•Carefullyplannedcoreneedle[preferred]orincisionalbiopsyafteradequateimaging(SeeSARC-D)pPlacebiopsyalongfutureresectionaxiswithminimaldissectionandcarefulattentiontohemostasispBiopsyshouldestablishgradeandhistologicsubtypecpAsappropriate,useancillarydiagnosticmethodologiesd•Imagingof•Additionalimagingasindicated;SeePrinciplesofImaging(SARC-A)•Thefollowingconditionsarelinkedtoincreasedincidenceofsarcomaandothercancers:pForpatientswithneurofibromatosis,fSeeNCCNGuidelinesforCentralNervousSystemCancers(PSCT-3)pForLi-Fraumenisyndrome,SeeNCCNGuidelinesforGenetic/FamilialHigh-RiskAssessment:Breast,Ovarian,andPanc