Dermatofibrosarcoma Protuberans of the Breast.

Dermatofibrosarcoma protuberans (DFSP) is a rare, low-grade, fibroblastic mesenchymal tumor derived from the dermis. Breast is an uncommon site with an incidence of only 0.8-4.5% and an overall population incidence at any site of 4.2-4.5 per million. Surgical excision with 2-3 cm margin is the gold standard treatment. Selected cases are subjected to radiotherapy or systemic therapy with Imatinib. Due to the rare presentation, we report a similar case of DFSP on the left breast in a 42-year-old woman, who was initially diagnosed with benign phyllodes tumor of the left breast and final histopathology report of the wide local excision specimen diagnosed DFSP of the breast.