A case is presented of dermatofibrosarcoma protuberans of the gluteal region with metastasis to the lung appearing 7 years after wide excision of the primary lesion. The world literature is reviewed. The clinical and pathologic features of dermatofibrosarcoma are reviewed and treatment is discussed, with the aim of emphasizing the need for long-term follow-up examination of lymph nodes and for metastases following wide excision of these lesions.
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