Multiple recurrent dermatofibrosarcoma protuberans of the hand

Summary We present a rare case of a dermatofibrosarcoma protuberans arising on the dorsum of the hand in a 51-year-old woman, who had experienced four recurrences of the tumour following local excisions. We performed a radical surgical excision of the lesion and covered the defect with a distal ulnar artery skin island flap. Adjuvant radiation therapy followed the surgical treatment. Forty months postoperatively, the patient has a functional hand without signs of recurrence and no evidence of disease. Wide surgical excision with margins of 2.5–3 cm is the optimal treatment for dermatofibrosarcoma protuberans. For selected patients, like those presenting large tumours involving the hand, adequate removal is not easily obtainable, or may result in major functional deficits. An alternative strategy may be the combination of less extensive surgery and radiotherapy, in order to prevent mutilation, decrease the local recurrence rates, and minimize the risk of metastases.