Recurrent dermatofibrosarcoma protuberans of the scalp followed by brain metastasis - A case report with literature review

Dermatofibrosarcoma protuberans (DFSP) is a fibrohistiocytic tumor of intermediate malignancy. It is characterized by a locally aggressive growth and a high rate of local recurrence. Metastases are very rare and the most common sites of metastasis are the lungs and lymph nodes. We report a 53-year-old female patient with a DFSP on the scalp. She developed local recurrence of DFSP 4 years after simple excision and right frontal lobe metastasis in the sixth year. The pathological examinations revealed dedifferentiation from low-grade DFSP to higher grade fibrosarcomatous features in the recurrent and metastatic lesions. Immunohistochemical studies showed diffuse CD34 expression in recurrent tumor and focal loss of CD34 expression associated with fibrosarcomatous change in the brain metastatic tumor. DFSP is not only locally invasive but also has a definite metastatic potential. The importance of complete local excision with close surveillance, especially for the patients with poor prognostic factors, should be emphasized.