Abstract

Dermatofibrosarcoma protuberans (DFSP) is a rare low-grade soft tissue sarcoma and the breast is less commonly affected than the torso. DFSP can be misdiagnosed as neurofibroma, desmoid tumor, malignant melanoma, or Kaposi sarcoma. A variant of DFSP originates from the subcutaneous layer and is called subcutaneous variant dermatofibrosarcoma protuberans (SC-DFSP). It can be easily confused with breast phyllodes tumor, myofibroblastoma, myoepithelioma, or advanced breast cancer, such as fibromatosis-like metastatic carcinoma. Following punch biopsy, the treatment of choice for breast DFSP is complete excision with negative margins ≥ 2 cm. We present a case of breast SC-DFSP with nipple areolar complex invasion.

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