軟組織肉瘤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≠M(fèi)offittCancerCenterJanaiCarr-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,MDT?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/oncologyTInternalmedicine?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