NCCNClinicalPracticeGuidelinesinOncologyNCCNGuidelines?)DermatofibrosarcomaProtuberansersionNovemberVersion1.2022,11/17/21?2021NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.PrintedbyMinTangon3/14/20227:31:17AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright?2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.uberansdexChrysalyneD.Schmults,MD,MS/Chair??Dana-Farber/BrighamandWomen’senterRachelBlitzblau,MD,PhD/ViceChair§DukeCancerInstituteSumairaZ.Aasi,MD?StanfordCancerInstituteMuradAlam,MD,MBA,MSCI??ζRobertH.LurieComprehensiveCancerCenterofNorthwesternUniversityJamesS.Andersen,MD??CityofHopeNationalMedicalCenterBrianC.Baumann,MD§SitemanCancerCenteratBarnes-JewishHospitalandWashingtonUniversitySchoolofMedicineJeremyBordeaux,MD,MPH?CaseComprehensiveCancerCenter/UniversityHospitalsSeidmanCancerCenterandClevelandClinicTaussignstitutePei-LingChen,MD,PhD≠M(fèi)offittCancerCenterRobertChin,MD,PhD§UCLAJonssonComprehensiveCancerCenterCarloM.Contreras,MD?TheOhioStateUniversityComprehensiveCancerCenter-JamesCancerHospitalandSoloveResearchInstituteDominickDiMaio,MD≠Fred&PamelaBuffettCancerCenternesPanelDisclosuresJessicaM.Donigan,MD?HuntsmanCancerInstituteattheUniversityofUtahJeffreyM.Farma,MD?FoxChaseCancerCenterMaxwellA.Fung,MD?≠UCDavisComprehensiveCancerCenterKarthikGhosh,MDTCancerCenterRoyC.Grekin,MD??UCSFHelenDillerFamilyComprehensiveCancerCenterKellyHarms,MD,PhD?UniversityofMichiganRogelCancerCenterAlanL.Ho,MD,PhD?MemorialSloanKetteringCancerCenterAshleyHolder,MD?O’NealComprehensiveCancerCenteratUABJohnNicholasLukens,MD§AbramsonCancerCenterattheUniversityofPennsylvaniaTheresaMedina,MD?UniversityofColoradoCancerCenterKishwerS.Nehal,MD??MemorialSloanKetteringCancerCenterPaulNghiem,MD,PhD?FredHutchinsonCancerResearchCenter/SeattleCancerCareAllianceSooPark,MD?UCSanDiegoMooresCancerCenterTejeshPatel,MD?≠St.JudeChildren’sResearchHospital/UniversityofTennesseeHealthScienceIgorPuzanov,MD,MSCI?RoswellParkComprehensiveCancerCenterJeffreyScott,MD,MHS?TheSidneyKimmelComprehensiveCancerCenteratJohnHopkinsAleksandarSekulic,MD,PhD?MayoClinicCancerCenterAshokR.Shaha,MD?ζMemorialSloanKetteringCancerCenterDivyaSrivastava,MD?UTSouthwesternSimmonsComprehensiveCancerCenterWilliamStebbins,MD??Vanderbilt-IngramCancerCenterValenciaThomas,MD?TheUniversityofTexasYaohuiG.Xu,MD,PhD?UniversityofWisconsineCancerCenterBDermatologyTInternalmedicine?MedicaloncologyζOtolaryngology≠Pathology/Dermatopathology?Reconstructivesurgery§Radiotherapy/Radiationoncology?Surgery/Surgicaloncology*DiscussionSectionWritingCommitteeVersion1.2022,11/17/21?2021NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.PrintedbyMinTangon3/14/20227:31:17AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright?2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.uberansdexlievesthatthebestlievesthatthebestmanagementforanypatientwithcancerisinaclinicaltrial.Participationinclinicaltrialsisespe-ciallyencouraged.FindanNCCNMemberInstitution:/home/member-institutions/.ofEvidenceandsusAllrecommendationsotherwiseindicated.ategoriesofEvidenceandConsensus.aryoftheGuidelinesUpdatesPresentationandWorkupDFSPentandFollowupDFSPPathologyDFSPAExcisionDFSPBiationTherapyDFSPCTheNCCNGuidelinesareastatementofevidenceandconsensusoftheauthorsregardingtheirviewsofcurrentlyacceptedapproachestotreatmentAnyclinicianseekingtoapplyorconsulttheNCCNGuidelinesisexpectedtouseindependentmedicaljudgmentinthecontextofindividualstancestodetermineanypatientscareortreatmentTheNationalComprehensiveCancerNetworkNCCNmakesnorepresentationsorwarrantiesofanykindregardingtheircontentuseorapplicationanddisclaimsanyresponsibilityfortheirapplicationoruseinanywayTheNCCNbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.?2021.Version1.2022,11/17/21?2021NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.UPDATESVersion1.2022,11/17/21?2021NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.PrintedbyMinTangon3/14/20227:31:17AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright?2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.uberansdexsionoftheNCCNGuidelinesforDermatofibrosarcomaProtuberansfromVersionincludenicalPresentationpWorkup,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).UPDATESVersion1.2022,11/17/21?2021NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.PrintedbyMinTangon3/14/20227:31:17AM.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.FSPforskincanceransiderpreoperativeMRIwithcontrastfortreatmentplanningifensivesubcutaneousextensionisctedPrintedbyMinTangon3/14/20227:31:17AM.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-1Version1.2022,11/17/21?2021NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.PrintedbyMinTangon3/14/20227:31:17AM.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-2Version1.2022,11/17/21?2021NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.PrintedbyMinTangon3/14/20227:31:17AM.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.Version1.2022,11/17/21?2021NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.DFSP-APrintedbyMinTangon3/14/20227:31:17AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright?2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.uberansdexPRINCIPLESOFEXCISIONEveryeffortshouldbemadetoachieveclearsurgicalmarginsCompletehistologicsurgicalmarginexaminationtoincludereviewofthepheralanddeepmarginisrecommendedwheneverpossibleTumorcharacteristicsincludelongirregularsubclinicalextensionsDebulkingspecimensfromallexcisionsshouldbeexaminedtoidentifyfibrosarcomatoustransformation(FS-DFSP)sincethisisassociatedwithmetastaticpotential.SurgicalApproach:MohsorOtherFormsofPDEMA?SeeNCCNGuidelinesforSquamousCellSkinCancer-PrinciplesofPDEMATechnique.?IfMohsorotherformsofPDEMAareunavailable,considerwideexcision.pReconstructionshouldbedelayeduntilclearmarginshavebeenverifiedtoavoidtheriskoftranslocatingtumorwithintheresectionbedmakingfurthermarginassessmentsinaccurate.Note:Allrecommendationsarecategory2Aunlessotherwiseindicated.ClinicalTrials:NCCNbelievesthatthebestmanagementofanypatientwithcancerisinaclinicaltrial.Participationinclinicaltrialsisespeciallyencouraged.DFSP-BVersion1.2022,11/17/21?2021NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.PrintedbyMinTangon3/14/20227:31:17AM.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-CVersion1.2022,11/17/21?2021NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.CAT-1Version1.2022,11/17/21?2021NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.PrintedbyMinTangon3/14/20227:31:17AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright?2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.uberansdexNCCNCategoriesofEvidenceandConsensusCategory1ategoryBaseduponhigh-levelevidence,thereisuniformNCCNconsensusthattheinterventionisappropriate.seduponlowerlevelevidencethereisuniformNCCNconsensusthattheinterventionisappropriateBaseduponlower-levelevidence,thereisNCCNconsensusthattheinterventionisappropriate.Baseduponanylevelofevidence,thereismajorNCCNdisagreementthattheinterventionisappropriate.Allrecommendationsarecategory2Aunlessotherwiseindicated.MS-1Version1.2022?2021NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelines?andthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCNPrintedbyMinTangon3/14/20227:31:17AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright?2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.NCCNGuidelinesVersion1.2022DermatofibrosarcomaProtuberans DiscussionDiscussionThisdiscussioncorrespondstotheNCCNGuidelinesforDermatofibrosarcomaProtuberans.Lastupdated:Dec16,2014.TableofContentsDiagnosisMS-2TreatmentMS-3Follow-upMS-4ReferencesMS-5MS-2Version1.2022?2021NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelines?andthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCNPrintedbyMinTangon3/14/20227:31:17AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright?2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.NCCNGuidelinesVersion1.2022DermatofibrosarcomaProtuberansOverviewDermatofibrosarcomaprotuberans(DFSP)isanuncommon,low-gradesarcomaoffibroblastoriginwithanincidencerateof4.2to4.5casespermillionpersonsperyearintheUnitedStates.1,2Itrarelymetastasizes.However,initialmisdiagnosis,prolongedtimetoaccuratediagnosis,andlargetumorsizeatthetimeofdiagnosisarecommon.Three-dimensionalreconstructionofDFSP3hasrevealedtumorswithhighlyirregularshapesandfrequentfinger-likeextensions.4Asaresult,incompleteremovalandsubsequentrecurrencearecommon.ThelocalrecurrencerateforDFSPinstudiesrangesfrom10%to60%,whereastherateofdevelopmentofregionalordistantmetastaticdiseaseisonly1%and4%to5%,respectively.5TheNCCNNonMelanomaSkinCancerPanelhasdevelopedtheseguidelinesoutliningthetreatmentofDFSPtosupplementtheirotherguidelinesNCCNGuidelinesforBasalCellan