Dermatofibrosarcoma protuberans (DFSP) is an uncommon dermal neoplasm that exhibits a high rate of local recurrence and infiltrative behavior, but has a low risk of metastasis. It arises as a slowly progressive, painless pink or violet plaque. Histologically, DFSP is characterized by a monomorphous spindle cell proliferation in a storiform pattern. The gold standard of treatment is surgical resection with negative margins. In cases where obtaining clear margins is not possible, radiation and systemic therapy with tyrosine kinase inhibitors, such as imatinib mesylate, has been shown to be effective.
Keywords: Cutaneous soft tissue sarcoma; Dermatofibrosarcoma protuberans; Fibrosarcomatous transformation; Imatinib mesylate; t(17;22) translocation.
Published by Elsevier Inc.