Dermatofibrosarcoma protuberans (DFSP) is a slowly growing fibrous tumor of the skin with a very high risk of local recurrence, but with low metastatic potential. Its malignant sarcomatous transformation with metastasis is exceptional. We report a rare case, of a 30-year-old patient, who presents dermatofibrosarcoma protuberans in the left supraclavicular region. Through this case we will underline the clinical presentation as well as the management of this benign but aggressive tumor.
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