隆突性纤维肉瘤2022.v2-NCCN（英文版）

NCCNClinicalPracticeGuidelinesinOncologyNCCNGuidelines®)DermatofibrosarcomaProtuberansersionMarchVersion2.2022,03/24/2022©2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.PrintedbyMinTangon3/26/20229:58:47AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.uberansdexChrysalyneD.Schmults,MD,MS/Chairϖ¶Dana-Farber/BrighamandWomen’senterRachelBlitzblau,MD,PhD/ViceChair§DukeCancerInstituteSumairaZ.Aasi,MDϖStanfordCancerInstituteMuradAlam,MD,MBA,MSCIϖ¶ζRobertH.LurieComprehensiveCancerCenterofNorthwesternUniversityJamesS.Andersen,MD¶ŸCityofHopeNationalMedicalCenterBrianC.Baumann,MD§SitemanCancerCenteratBarnes-JewishHospitalandWashingtonUniversitySchoolofMedicineJeremyBordeaux,MD,MPHϖCaseComprehensiveCancerCenter/UniversityHospitalsSeidmanCancerCenterandClevelandClinicTaussignstitutePei-LingChen,MD,PhD≠MoffittCancerCenterRobertChin,MD,PhD§UCLAJonssonComprehensiveCancerCenterCarloM.Contreras,MD¶TheOhioStateUniversityComprehensiveCancerCenter-JamesCancerHospitalandSoloveResearchInstituteDominickDiMaio,MD≠Fred&PamelaBuffettCancerCenternesPanelDisclosuresJessicaM.Donigan,MDϖHuntsmanCancerInstituteattheUniversityofUtahJeffreyM.Farma,MD¶FoxChaseCancerCenterKarthikGhosh,MDÞCancerCenterRoyC.Grekin,MDϖ¶UCSFHelenDillerFamilyComprehensiveCancerCenterKellyHarms,MD,PhDϖUniversityofMichiganRogelCancerCenterAlanL.Ho,MD,PhD†MemorialSloanKetteringCancerCenterJohnNicholasLukens,MD§AbramsonCancerCenterattheUniversityofPennsylvaniaTheresaMedina,MD†UniversityofColoradoCancerCenterKishwerS.Nehal,MDϖ¶MemorialSloanKetteringCancerCenterPaulNghiem,MD,PhDϖFredHutchinsonCancerResearchCenter/SeattleCancerCareAllianceKellyOlino,MD¶YaleCancerCenter/SmilowCancerHospitalSooPark,MD†UCSanDiegoMooresCancerCenterTejeshPatel,MDϖ≠St.JudeChildren’sResearchHospital/UniversityofTennesseeHealthScienceCenterIgorPuzanov,MD,MSCI†RoswellParkComprehensiveCancerCenterJeffreyScott,MD,MHSϖTheSidneyKimmelComprehensiveCancerCenteratJohnHopkinsAleksandarSekulic,MD,PhDϖMayoClinicCancerCenterAshokR.Shaha,MD¶ζMemorialSloanKetteringCancerCenterDivyaSrivastava,MDϖUTSouthwesternSimmonsComprehensiveCancerCenterValenciaThomas,MDϖTheUniversityofTexasYaohuiG.Xu,MD,PhDϖUniversityofWisconsineCancerCenterMehranYusuf,MD§O'NealComprehensiveCancerCenteratUABenPhDMcCulloughRNBSBDermatologyÞInternalmedicine†MedicaloncologyζOtolaryngology≠Pathology/DermatopathologyŸReconstructivesurgery§Radiotherapy/Radiationoncology¶Surgery/Surgicaloncology*DiscussionSectionWritingCommitteeVersion2.2022,03/24/2022©2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.PrintedbyMinTangon3/26/20229:58:47AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.uberansdexlievesthatthebestlievesthatthebestmanagementforanypatientwithcancerisinaclinicaltrial.Participationinclinicaltrialsisespe-ciallyencouraged.FindanNCCNMemberInstitution:/home/member-institutions/.ofEvidenceandsusAllrecommendationsotherwiseindicated.ategoriesofEvidenceandConsensus.aryoftheGuidelinesUpdatesPresentationandWorkupDFSPentandFollowupDFSPPathologyDFSPAExcisionDFSPBiationTherapyDFSPCTheNCCNGuidelinesareastatementofevidenceandconsensusoftheauthorsregardingtheirviewsofcurrentlyacceptedapproachestotreatmentAnyclinicianseekingtoapplyorconsulttheNCCNGuidelinesisexpectedtouseindependentmedicaljudgmentinthecontextofindividualstancestodetermineanypatientscareortreatmentTheNationalComprehensiveCancerNetworkNCCNmakesnorepresentationsorwarrantiesofanykindregardingtheircontentuseorapplicationanddisclaimsanyresponsibilityfortheirapplicationoruseinanywayTheNCCNbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.©2021.Version2.2022,03/24/2022©2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.UPDATESVersion2.2022,03/24/2022©2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.PrintedbyMinTangon3/26/20229:58:47AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.uberansdexsionoftheNCCNGuidelinesforDermatofibrosarcomaProtuberansfromVersioninclude•TheDiscussionsectionhasbeenupdatedtoreflectthechangesinthealgorithm.sionoftheNCCNGuidelinesforDermatofibrosarcomaProtuberansfromVersionincludenicalPresentationpWorkup,fourthbulletrevised:ConsiderpreoperativeMRIwithcontrastfortreatmentplanningifextensiveextracutaneoussubcutaneousextensionorarecurrenttumorissuspected.•Footnoteb,lastsentenceremoved:Biopsyclosuresareencouragedtobekeptsmallsoasnottodistorttheanatomyfordefinitiveexcision.•Footnotedrevised:Iffibrosarcomatouschanges/malignanttransformationsareisnotedfound,multidisciplinaryconsultationforconsiderationoffurthertreatmentandsurveillanceisrecommended.SeetheNCCNGuidelinesforSoftTissueSarcomaformultimodaltherapyandsurveillanceconsiderations.Multidisciplinaryconsultationisrecommendedforotherhigh-riskfeatures.(AlsopageDFSP-2)DFSP-2•TreatmentpHeaderrevised:ExcisionwithMohsmicrographicsurgery(MMS)orotherformsofCCPDMAperipheralanddeepenfacemarginassessment(PDEMA).•RevisedfootnotespFootnotee:Themostcommonlyusedformofcompletecircumferentialperipheralanddeepmarginassessment(CCPDMA)PDEMAisMohs(MMS).SeeNCCNGuidelinesforSquamousCellSkinCancer-PrinciplesofCCPDMAPDEMATechnique.IfCCPDMAisunavailable,.theriskofconcealingresidualtumorbelowimmobilizedtissue.SeePrinciplesofExcision(DFSP-B).Whenanatomicstructuresatthedeepmargin(eg,majorvessels,nerves,bone)precludecompletehistologicevaluationofthemarginalsurfaceviaMohsorotherformsofPDEMA,MohsorotherformsofPDEMAshouldbeusedtoevaluateasmuchofthemarginalsurfaceasfeasible.Treatmentconsiderationsfornon-visualizedareasmaybethesubjectofmultidisciplinarydiscussion.pFootnoteg:Considerneoadjuvantimatinibforpatientsinwhomresectionwithnegativemarginsmayresultinunacceptablefunctionalorcosmeticoutcomes.isnotanticipatedtoachievenegativemarginswithoutunacceptablefunctionalorcosmeticoutcomes.UgurelS,etal.ClinCancerRes2014;20:499-510.pFootnotej:FornegativemarginsWhenMohsorotherformsofPDEMAareutilized,RTisnotrecommended.WhenMohsorotherformsofPDEMAarenotutilized,considerRTifmarginsare<1cm.RTcanbeconsideredfortreatmentofpositivemarginsifnotgivenpreviouslyandfurtherresectionisnotfeasible.pFootnotek:Tumorslackingthet(17;22)rearrangementmaynotrespondtoimatinib.Cytogeneticanalysis(molecularorconventional)ofatumormaybeusefulpriortotheinstitutionofimatinibtherapy.Navarrete-DechentC,etal.JAMADermatol2019;155:361-369.•Footnotefadded:IfPDEMAisunavailable,considerwideexcision.Wideunderminingisdiscouragedpriortoconfirmationofclearmarginsduetothedifficultyofinterpretingsubsequentre-excisedmargins,andtheriskofconcealingresidualtumorbelowmobilizedtissue.SeePrinciplesofExcision(DFSP-B).UPDATESVersion2.2022,03/24/2022©2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.PrintedbyMinTangon3/26/20229:58:47AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.uberansdexsionoftheNCCNGuidelinesforDermatofibrosarcomaProtuberansfromVersioninclude•PrinciplesofPathologypFirstbulletrevised:Evaluationbyaqualifiedphysicianwithspecificexpertiseinsarcoma/softtissuepathologyordermatopathologyispreferred(ifavailable).pFourthbulletrevised:Fibrosarcomatoustransformation(FS-DFSP)isreflectedbycharacterizedbytransitionfromstoriformtoaherringbonepattern,withahigherdegreeofcellularity,cytologicatypia,mitoticactivity(>5/10high-powerfields[HPF]),andnegativefrequentlossofCD34immunostaining.pSixthbulletrevised:Margincontrolduringexcision(seePrinciplesofExcision)mayrequireoccasionallybeaidedbyH&EsectionssupplementedbyCD34immunohistochemistry.•Footnote2revised:FS-DFSPshouldbenotedwhenpresentasthemetastaticriskis15%–20%andthepatientshouldbereferredtoacenterwithexpertiseinmanagementofsofttissuesarcomas.asitisassociatedwithapoorprognosis.DFSP-B•PrinciplesofExcisionpGoal,bulletrevised:Everyeffortshouldbemadetoachieveclearsurgicalmargins.Completehistologicsurgicalmarginexaminationtoincludereviewoftheentireexcisedperipheralanddeepmarginisrecommended,wheneverpossible.Tumorcharacteristicsincludelong,irregular,subclinicalextensions.DebulkingSspecimensfromdebulking/Mohsallexcisionsshouldbeexaminedtoidentifyfibrosarcomatoustransformation(FS-DFSP)sincethisisassociatedwithmetastaticpotential.pSurgicalapproach,headingrevised:MohsMicrographicSurgeryorOtherFormsofCCPDMAPDEMA.◊Firstbulletrevised:SeeNCCNGuidelinesforSquamousCellSkinCancer-PrinciplesofCCPDMAPDEMATechnique.◊Secondbulletrevised:IfCCPDMAMohsorotherformsofPDEMAareunavailable,considerwideexcision.–Firstsub-bulletremoved:Ifthereisconcernthatthesurgicalmarginsarenotcompletelyclear,consideravoidingrepairwithaflaporothertechnique,asitmayimpedemonitoringofthesiteanddelaydetectionofarecurrence.Split-thicknessskingrafting(STSG)maybeconsidered.–Secondsub-bulletremoved:Itisrecommendedthatanyreconstructioninvolvingextensiveunderminingortissuemovementbeavoidedordelayeduntilnegativehistologicmarginsareverifiedtopreventsubclinicaltumorpersistence,particularlyinadvertentconcealmentofresidualtumorbelowrepositionedtissueorarepair.–Newsub-bulletadded:Reconstructionshouldbedelayeduntilclearmarginshavebeenverifiedtoavoidtheriskoftranslocatingtumorwithintheresectionbedmakingfurthermarginassessmentsinaccurate.DFSP-C•PrinciplesofRTpGeneralTreatmentInformation,AdjuvantRT,Negativemargins:◊Firstsub-bulletrevised:WhenMohsorotherformsofPDEMAareutilized,RTisnotrecommended.◊Newsub-bulletadded:WhenMohsorotherformsofPDEMAarenotutilized,considerRTifmarginsare<1cm.FSPforskincanceransiderpreoperativeMRIwithcontrastfortreatmentplanningifensivesubcutaneousextensionisctedPrintedbyMinTangon3/26/20229:58:47AM.ForpersonaluseFSPforskincanceransiderpreoperativeMRIwithcontrastfortreatmentplanningifensivesubcutaneousextensioniscteduberansdexCLINICALPRESENTATIONWORKUPHPybcybcpHematoxylinandeosin(H&E)pImmunopanel(eg,CD34,factorXIIIa)eorotherhigheorotherhighriskfeaturesdAsdecisionsaboutdiagnosisandresectionmaybemultidisciplinaryltationatacenterwithcializedexpertiseshouldonsideredespeciallyforlargeoraFormoreinformation,seeAmericanAcademyofDermatologyAssociation.bThistumorisfrequentlymisdiagnosedduetoinadequatetissuesampling/superficialbiopsy.Punch,incisional,orcorebiopsy,preferablyincludingthedeepersubcutaneouslayer,isstronglyrecommendedforsufficienttissuesamplingandaccuratepathologicassessment.Ifbiopsyisindeterminateorclinicalsuspicionremains,rebiopsyisrecommended.Wideunderminingisdiscouragedduetothedifficultyofinterpretingsubsequentre-excisionspathologically.cPrinciplesofPathology(DFSP-A).dIffibrosarcomatoustransformationisfound,multidisciplinaryconsultationforconsiderationoffurthertreatmentandsurveillanceisrecommended.SeetheNCCNGuidelinesforSoftTissueSarcomaformultimodaltherapyandsurveillanceconsiderations.Note:Allrecommendationsarecategory2Aunlessotherwiseindicated.ClinicalTrials:NCCNbelievesthatthebestmanagementofanypatientwithcancerisinaclinicaltrial.Participationinclinicaltrialsisespeciallyencouraged.DFSP-1Version2.2022,03/24/2022©2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.PrintedbyMinTangon3/26/20229:58:47AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.uberansdexTREATMENTsurgicalsurgicalwithMohsorotherformsofperipheralanddeepenfacemargin(PDEMA)(PDEMA)d,e,f,gsurgeryNegativeuntilmarginsionefuntilmarginsPositiveatmentAatmentTREATMENTObservationMultidisciplinaryconsultationforofRTofRTh,jvs.dIffibrosarcomatoustransformationisfound,multidisciplinaryconsultationforconsiderationoffurthertreatmentandsurveillanceisrecommended.SeetheNCCNGuidelinesforSoftTissueSarcomaformultimodaltherapyandsurveillanceconsiderations.eThemostcommonlyusedformofPDEMAisMohs.SeeNCCNGuidelinesforSquamousCellSkinCancer-PrinciplesofPDEMATechnique.Whenanatomicstructuresatthedeepmargin(eg,majorvessels,nerves,bone)precludecompletehistologicevaluationofthemarginalsurfaceviaMohsorotherformsofPDEMA,MohsorotherformsofPDEMAshouldbeusedtoevaluateasmuchofthemarginalsurfaceasfeasible.Treatmentconsiderationsfornon-visualizedareasmaybethesubjectofmultidisiplinarydiscussion.fIfPDEMAisunavailable,considerwideexcision.Wideunderminingisdiscouragedpriortoconfirmationofclearmarginsduetothedifficultyofinterpretingsubsequentre-excisedmargins,andtheriskofconcealingresidualtumorbelowmobilizedtissue.SeePrinciplesofExcision(DFSP-B).FOLLOW-UP•Physicalexamwithfocusonprimarysiteonthsievery6–onthsittionoutregularxamTHERAPYFORRECURRENCE/METASTASISerredeferredefRThjifnotgivenpreviouslysectionnotfeasibleatinibkswherediseasenresectableRThjifnotgivenpreviouslysectionnotfeasibleconsultationlconsultationlgConsiderneoadjuvantimatinibforpatientsinwhomresectionwithnegativemarginsmayresultinunacceptablefunctionalorcosmeticoutcomes.UgurelS,etal.ClinCancerRes2014;20:499-510.hSeePrinciplesofRadiationTherapy(DFSP-C).iMRIwithcontrastmaybehelpfultodetectearlyrecurrenceinpatientswithhigh-risklesionsordelineatetumorextentwhenphysicalexamisinsufficientorunreliable.jWhenMohsorotherformsofPDEMAareutilized,RTisnotrecommended.WhenMohsorotherformsofPDEMAarenotutilized,considerRTifmarginsare<1cm.RTcanbeconsideredfortreatmentofpositivemarginsifnotgivenpreviouslyandfurtherresectionisnotfeasible.kNavarrete-DechentC,etal.JAMADermatol2019;155:361-369.lSeeNCCNGuidelinesforSTAGEIVSoftTissueSarcoma(EXTSARC-5).Note:Allrecommendationsarecategory2Aunlessotherwiseindicated.ClinicalTrials:NCCNbelievesthatthebestmanagementofanypatientwithcancerisinaclinicaltrial.Participationinclinicaltrialsisespeciallyencouraged.DFSP-2Version2.2022,03/24/2022©2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.PrintedbyMinTangon3/26/20229:58:47AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.uberansdexPRINCIPLESOFPATHOLOGY1•Evaluationbyaqualifiedphysicianwithspecificexpertiseinsarcoma/softtissuepathologyordermatopathologyispreferred(ifavailable).•Thespindledcellsarrangedinastoriformorfascicularpatternaretypicallyblandwithminimalcytologicatypia.•ImmunohistochemistryforCD34ismostlypositive,andfactorXIIIanegative.•Fibrosarcomatoustransformation(FS-DFSP)ischaracterizedbytransitionfromstoriformtoaherringbonepattern,withahigherdegreeofcellularitycytologicatypiamitoticactivityhighpowerfieldsHPFsandfrequentlossofCD34immunostaining.2Forequivocallesionsconsiderfluorescenceinsituhybridization(FISH),polymerasechainreaction(PCR),orconventionalcytogeneticstodetecttqqwhichisahallmarkofDFSPFusionofthecollagentypeIalphageneCOLAat17q22,withtheplatelet-derivedormtheoncogenicchimericfusiongeneCOLAPDGFMargincontrolduringexcisionseePrinciplesofExcision[DPSP-B])mayoccasionallybeaidedbyH&EsectionssupplementedbyCD34istry1Currently,noAmericanJointCommitteeonCancer(AJCC)orCollegeofAmericanPathologists(CAP)synopticreportingisdefined.2FS-DFSPshouldbenotedwhenpresentasthemetastaticriskis15%–20%andthepatientshouldbereferredtoacenterwithexpertiseinmanagementofsofttissuesarcomas.Note:Allrecommendationsarecategory2Aunlessotherwiseindicated.ClinicalTrials:NCCNbelievesthatthebestmanagementofanypatientwithcancerisinaclinicaltrial.Participationinclinicaltrialsisespeciallyencouraged.Version2.2022,03/24/2022©2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.DFSP-APrintedbyMinTangon3/26/20229:58:47AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.uberansdexPRINCIPLESOFEXCISIONEveryeffortshouldbemadetoachieveclearsurgicalmarginsCompletehistologicsurgicalmarginexaminationtoincludereviewofthepheralanddeepmarginisrecommendedwheneverpossibleTumorcharacteristicsincludelongirregularsubclinicalextensionsDebulkingspecimensfromallexcisionsshouldbeexaminedtoidentifyfibrosarcomatoustransformation(FS-DFSP)sincethisisassociatedwithmetastaticpotential.SurgicalApproach:MohsorOtherFormsofPDEMA•SeeNCCNGuidelinesforSquamousCellSkinCancer-PrinciplesofPDEMATechnique.•IfMohsorotherformsofPDEMAareunavailable,considerwideexcision.pReconstructionshouldbedelayeduntilclearmarginshavebeenverifiedtoavoidtheriskoftranslocatingtumorwithintheresectionbedmakingfurthermarginassessmentsinaccurate.Note:Allrecommendationsarecategory2Aunlessotherwiseindicated.ClinicalTrials:NCCNbelievesthatthebestmanagementofanypatientwithcancerisinaclinicaltrial.Participationinclinicaltrialsisespeciallyencouraged.DFSP-BVersion2.2022,03/24/2022©2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.PrintedbyMinTangon3/26/20229:58:47AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.uberansdexPRINCIPLESOFRADIATIONTHERAPYrmationuvantRTpPositiveMargins/GrossDisease◊50–60Gyforindeterminateorpositivemargins,andupto66Gyforpositivemarginorgrosstumor(2-Gyfractionsperday).◊Fieldstoextendwidelybeyondsurgicalmargin(eg,3–5cm)whenclinicallyfeasible.pNegativeMargins◊WhenMohsorotherformsofPDEMAareutilized,RTisnotrecommended.◊WhenMohsorotherformsofPDEMAarenotutilized,considerRTifmarginsare<1cm.•Recurrence/Metastasis:pRTifnotgivenpreviouslyandfurtherresectionisnotfeasible;50–60Gyforindeterminateorpositivemargins,andupto66Gyforpositivemarginorgrosstumor(2-Gyfractionsperday).pFieldstoextendwidelybeyondsurgicalmargin(eg,3–5cm)whenclinicallyfeasible.Note:Allrecommendationsarecategory2Aunlessotherwiseindicated.ClinicalTrials:NCCNbelievesthatthebestmanagementofanypatientwithcancerisinaclinicaltrial.Participationinclinicaltrialsisespeciallyencouraged.DFSP-CVersion2.2022,03/24/2022©2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.CAT-1Version2.2022,03/24/2022©2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.PrintedbyMinTangon3/26/20229:58:47AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.uberansdexNCCNCategoriesofEvidenceandConsensusCategory1ategoryBaseduponhigh-levelevidence,thereisuniformNCCNconsensusthattheinterventionisappropriate.seduponlowerlevelevidencethereisuniformNCCNconsensusthattheinterventionisappropriateBaseduponlower-levelevidence,thereisNCCNconsensusthattheinterventionisappropriate.Baseduponanylevelofevidence,thereismajorNCCNdisagreementthattheinterventionisappropriate.Allrecommendationsarecategory2Aunlessotherwiseindicated.MS-1Version2.2022©2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelines®andthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCNPrintedbyMinTangon3/26/20229:58:47AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.NCCNGuidelinesVersion2.2022DermatofibrosarcomaProtuberans DiscussionDiscussionThisdiscussioncorrespondstotheNCCNGuidelinesforDermatofibrosarcomaProtuberans.Lastupdated:January31st,2022.TableofContentsOverviewMS-2LiteratureSearchCriteriaandGuidelinesUpdateMethodologyMS-2EvaluationMS-2TreatmentMS-3Follow-upMS-4ReferencesMS-6MS-2Version2.2022©2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelines®andthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCNPrintedbyMinTangon3/26/20229:58:47AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.NCCNGuidelinesVersion2.2022DermatofibrosarcomaProtuberansOverviewofibrosarcomaprotuberansDFSPisanuncommonlowgradesarcomaoffibroblastoriginwithanincidencerateof4.1to4.5casespermillionpersonsperyearintheUnitedStates.1-4ApredilectionforoccurringinAfricanAmericanshasbeenreportedinonestudy.3Initialmisdiagnosisprolongedtimetoaccuratediagnosis,andlargetumorsizeatthetimeofdiagnosisarecommon.However,itrarelymetastasizes.5Whenmetastasisoccurs,itistypicallyinthelung,bone,orregionallymphnodes.Three-dimensionalreconstructionofDFSP6hasrevealedtumorswithhighlyirregularshapesandfrequentfinger-likeextensions.7Asaresult,incompleteremovalandsubsequentrecurrencearecommonwithoutattentiontofullassessmentoftheperipheralanddeepmarginThelocalrecurrencerateforwidelocalexcisionWLEofDFSPinstudiesrangesfrom10%to60%,whereastherateofdevelopmentofregionalordistantmetastaticdiseaseisonly1%and4%to7.4%,respectively.8,9LiteratureSearchCriteriaan