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[New molecular approaches in dermatofibrosarcoma protuberans].

Dermatofibrosarcoma protuberans (DFSP) is a low-grade malignancy of the skin and subcutaneous tissues that only rarely forms distant metastases. More than 90% of cases are associated with a chromosomal translocation involving the COL1A1 gene on chromosome 17 and the PDGFB gene on chromosome 22. Management of this disease is primarily surgical with excellent rates of local control obtained using either wide local excision or Mohs micrographic surgery. Data have recently shown that inhibiting platelet-derived growth factor receptors (PDGFR) with imatinib mesylate can induce high rates of clinical response in patients with unresectable or metastatic DFSP. Although wide surgical excision remains standard care, patients with locally advanced disease not suitable for surgical excision can be treated with imatinib mesylate, which sometimes allows residual DFSP to be surgically excised.