Dermatofibrosarcoma Protuberans

Dermatofibrosarcoma protuberans (DFSP) is a rare malignant cutaneous tumor with an annual incidence of 4.2 per million. The tumor typically grows slowly and has a proclivity toward local invasiveness and away from distant metastasis. The mainstay of treatment is complete surgical resection, which is curative in almost all cases. The tumor’s characteristics make this difficult, however, and reexcision to negative margins can be challenging. DFSP can also present with or develop fibrosarcomatous change (DFSP-FS), a more aggressive variant. Thus, these tumors require thoughtful management. In this review, we discuss the biologic background of DFSP, describe the tumor’s behavior and presentation, and discuss current concepts in management.   This review contains 5 figures, 4 tables and 28 references Key words: dermatofibrosarcoma protuberans, fibrosarcomatous change, imatinib, local recurrence, micrographic surgery, nonmelanoma, radiation, sarcoma, skin cancer, surgical excision