Dermatofibrosarcoma protuberans (DFSP), a rare low-grade mesenchymal subcutaneous neoplasm, is characterized by a propensity for local recurrence in up to 50% of patients but has a low metastatic potential. The reported incidence of metastases in a large series was 0-9%. The metastasis is usually a late event and often follows local recurrences. The presence of fibrosarcomatous areas (FS) within DFSP (DFSP-FS) increases the risk for local recurrence and metastases. We report a patient who developed metastatic disease in the lungs 6 months after complete excision of DFSP-FS of the thigh without local recurrence.
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