Dermatofibrosarcoma protuberans is an intermediate-grade sarcoma that arises in the dermis of the trunk, proximal extremities, and less commonly, in the head and neck area of adults. Dermatofibrosarcoma protuberans has a deceptively innocuous early clinical appearance and generally exhibits an indolent course for years before entering a rapid growth phase. Its locally invasive behavior results in early recurrence if prompt, wide excision is not performed. Dermatofibrosarcoma protuberans rarely metastasizes through the bloodstream or, less often, to locoregional lymph nodes after multiple local recurrences. Fibrosarcomatous areas within the tumor result in a more aggressive course. Although wide excision is the mainstay of therapy, recent trials using the Mohs' surgical approach suggest that this technique may have a role in the future treatment of this tumor.

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