Dermatofibrosarcoma protuberans of the breast: A case study

Abstract

Dermatofibrosarcoma protuberans (DFSP) is a superficial mesenchymal neoplasm that originates from the dermal fibroblasts and tends to be locally aggressive. Although infrequent, it is one of the most common cutaneous sarcomas. It mainly affects young and middle-aged patients 20 to 50 years old. Any area of skin may be involved, but the most common sites of presentation are the trunk and extremities. DFSP of the breast is extremely rare. It classically presents as a nodular, exophytic, cutaneous mass, though initially it can manifest as a flat plague and can show persistent but slow growth for many years. Due to increased risk of local recurrence, the standard of care for localized disease is surgical excision with adequate margins. Wide local excision is the most common technique used, but as an alternative Mohs micrographic surgery has emerged as a procedure that offers lower local recurrence rates. Metastases are rare but have been previously reported. In such patients, treatment with imatinib or radiotherapy can be considered. The current case presents a 52-year-old lady with DFSP of the breast that was successfully managed by the Breast Unit of Athens Medical Center-Psychiko Clinic.

Keywords: breast lesion; dermatofibrosarcoma protuberans; soft tissue tumors; spindle cell tumors.

Figure 1

Histological and Immunohistochemical analysis of the resected tumor. (A) Hematoxylin-eosin staining with diffuse infiltration of subcutaneous fat. Magnification, x4. (B) Hematoxylin-eosin staining with storiform or whorled growth pattern, minimal cytological atypia and low mitotic activity. Magnification, x40. (C) Immunohistochemical analysis with CD34 positive (+) staining in spindled tumor cells (Magnification, x20) and (D) Immunohistochemical analysis with Ki67 (Mib-1) positive (+) in ~10% of tumor cells (Magnification, x20).