Dermatofibrosarcoma protuberans

Abstract

Dermatofibrosarcoma protuberans is the most common skin sarcoma, although its incidence is very low compared with other skin tumors. It presents as a slow-growing indurated plaque on which nodules develop over time. The lesion arises in the dermis but can invade subcutaneous tissue, fascia, muscle and even bone. COL1A1-PDGFB translocation is specific to dermatofibrosarcoma protuberans, and the presence of this fusion contributes to diagnosis in certain cases. A review of the literature provides evidence that recurrence is much lower after Mohs micorgraphic surgery than after conventional wide local excision. In the case of metastatic disease or when surgery would be mutilating, another recently approved treatment is the tyrosine kinase inhibitor imatinib.