Dermatofibrosarcomaprotuberans(DFSP),themostcommondermalsarcoma,isalow-grade,slowgrowingfibroblasticmalignantneoplasmthatmostfrequentlyaffectsmiddleagedadultsandischaracterizedbyahighlocalrecurrencerateandalowpropensityformetastasis.WidesurgicalresectionorMohsmicrographicsurgery(MMS)arethepreferredapproachesforlocalizeddisease,whileradiationtherapyiswarrantedforinoperablediseaseorforcaseswithpositivemarginswherere-excisionisnotpossible.DFSPisgenerallyregardedasrefractorytoconventionalchemotherapy.Treatmentoptionsforsystemicdiseasewerelimiteduntilthediscoveryofauniquetranslocation,t(17;22)(q22;q13)(COL1A1;PDGFB)foundinamajorityofcases.Inrecentyears,imatinib,aPDGFβR,ABLandKITinhibitor,hasrevolutionizedsystemictherapyinDFSP.Inthisreview,wesummarizetheepidemiological,clinical,histologicalandgeneticcharacteristicsofDFSPandupdatethereadersonitscurrentmanagement.
