Dermatofibrosarcoma Protuberans

Excerpt

Dermatofibrosarcoma protuberans (DFSP) is a rare soft tissue tumor that involves the dermis, subcutaneous fat, and in rare cases, muscle and fascia. The tumor typically presents as a slowly growing, firm plaque on the trunk of young adults. The cause of dermatofibrosarcoma protuberans is not clearly understood. Studies have implicated a chromosomal translocation, resulting in the fusion protein COL1A1-PDGFB, which promotes tumor growth through the overproduction of platelet-derived growth factor (PDGF). Diagnosis is made via skin biopsy. Dermatofibrosarcoma protuberans is considered an intermediate-grade malignancy with a low likelihood of metastasis but a high local recurrence rate. Given its propensity for a subclinical extension, the optimal treatment modality for dermatofibrosarcoma protuberans is Mohs micrographic surgery (MMS), a surgical technique that allows complete margin assessment and tissue preservation. Alternatively, dermatofibrosarcoma protuberans can be treated with wide local excision. The chemotherapeutic agent imatinib mesylate is currently FDA-approved for adults with unresectable, recurrent, or metastatic dermatofibrosarcoma protuberans.