Fibrosarcomatous dermatofibrosarcoma protuberans: a rapidly growing 30 cm mass on the posterior scalp

Dermatofibrosarcoma protuberans (DFSP) is a rare, slow-growing sarcoma of the skin and subcutaneous tissue, accounting for around 5 cases per million per year. Fibrosarcomatous transformation of DFSP occurs in 10%–15% of DFSP cases, with a higher risk of local recurrence, metastasis, and death. We present a case of a male in his 30s with a complaint of rapidly progressive mass in the occipital region of the head. Within 1 year, the mass enlarged by >30 cm. Physical examination revealed a skin-colored 40×30 cm mass with an overlying skin necrosis at the posterior scalp. Brain, neck, and chest computed tomography (CT) scans were performed. The mass was surgically excised by wide excision with a 2 cm margin. Pathological report confirmed fibrosarcomatous DFSP Grade 3 with decreased CD34 expression. Delayed reconstruction of free flap and split-thickness skin graft were subsequently performed. No recurrence was detected 3 months postoperatively.