Dermatofibrosarcoma Protuberans of Breast

Dermatofibrosarcoma protuberans (DFSP) is a rare malignant neoplasm of skin, subcutaneous tissue and dermis, with more potential of local infiltration, appears most frequently on the trunk, followed by extremities, head, neck, and extremely rare in breast. It often occurs in young adults and middle-aged adults. The standard of treatment for this disease is local surgical excision with 2-3 cm wide margins. We present a case of 49-year lady with right breast well-circumscribed, firm, tender, mobile, and progressive lump that was benign-looking, on physical examination as well as on mammography. She underwent local surgical resection and diagnosed as DFSP on microscopic and immunohistochemical analysis with positive deep margin. She was referred for further multidisciplinary management. Case was discussed in tumour board meeting and planned for re-resection of involved deep margin. After re-resection, she has remained disease-free for last 3 years without any evidence of local or distant recurrence. In this case report, the importance of surgical resection with adequate margins, is highlighted and long term follow-up by physical examination and ultrasound for any local recurrence of DFSP in breast.