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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.
DFSP usually has a long slow indolent course, with early tumors appearing as painless areas of cutaneous thickening. They may have pink, dark red, or even bluish discoloration, particularly at its periphery. Over time, they develop into a larger nodular mass, and ultimately can develop into a large fungating lesion. When they grow into the epidermal layer of the skin, they may eventually ulcerate. Unlike tumors of the subcutaneous tissue, DSPF is adherent or intimate with its overlying skin. Typically it is not adherent to underlying structures, with most tumors being superficial and less than 5 cm in size at time of diagnosis.
The duration of tumor growth ranges from months to years and may, in some cases, span decades. DFSP often is mistaken for lipomas, deep-seated epidermal cysts, scars, hypertrophic scars, keloid, dermatofibromas, nodular fasciitis, and insect bites and a delayed diagnosis is not uncommon. The trunk is the most common location (47%), followed by lower extremity (20%), upper extremity (18%), and finally head and neck (14%).
DFSP is a malignant tumor, but only metastasizes 1-4% of the time. Metastasis is a late clinical outcome and typically occurs only after several local recurrences.
Dermatofibrosarcoma Protuberans
