Dermatofibrosarcoma protuberans of the breast: A rare diagnosis requiring aggressive initial surgical therapy

Abstract

Dermatofibrosarcoma protuberans (DFSP) is an extremely rare cutaneous neoplasm of the dermal layer of the skin and is histologically classified as a sarcoma. DFSP itself has an incidence of 0.8 cases per million annually. It accounts for 1% of all soft tissue sarcomas and less than 0.1% of all malignancies. DFSP has a high rate of local recurrence especially if there are positive margins on excision, but only up to 4% develop metastasis. Most reported cases are located in the trunk, extremities and head especially scalp. However, DFSP of the breast is very rare with very few reported in the literature. We report a case of a 33-year-old woman who presented with a histological diagnosis of DFSP of the breast, based on incisional biopsy. We report this case which highlights the important aspects in evaluating DFSP in the breast as well as its treatment.