Dermatofibrosarcoma protuberans

Dermatofibrosarcoma protuberans (DFSP) is a rare neoplasm of the dermis layer of the skin, and is classified as a sarcoma. In many respects, the disease behaves as a benign tumor, but in 2-5% of cases it can metastasize, so it should be considered to have malignant potential.

Over 95% of DFSP tumors have the chromosomal translocation t(17;22), which results in over-expression of platelet-derived growth factor receptor B (PDGFB).

Treatment is primarily surgical, with chemotherapy and radiation therapy sometimes being used.

There is clinical evidence that imatinib, which inhibits PDGFB, may be effective for tumors positive for the t(17;22) translocation.