Dermatofibrosarcoma Protruberans of the Breast Skin Simulating Mammary Carcinoma

Dermatofibrosarcoma protuberans is a rare soft tissue sarcoma that arises from the dermis and invades deeper tissues. Usually, it behaves as an intermediate grade malignancy but in rare instances, it can metastasize. Frequent occurrences have been observed in trunk and extremities but involvement of the breast has rarely been reported. Therefore, dermatofibrosarcoma protuberans (DFSP) breast often masquerades primary breast malignancy on clinical and radiological grounds. Histomorphology and immunohistochemistry are helpful in making a definitive diagnosis. In the present case, monomorphic spindle cells were seen arranged in classical storiform pattern with large areas of myxoid change. Many times, such myxoid areas pose a diagnostic challenge and necessitate differentiation from other myxoid neoplasms. Herein, immunohistochemistry offers an advantage where tumor cells show strong reactivity for CD34 antibody. Wide surgical excision remains the treatment of choice. The salient clinical features, histogenesis, histomorphology, and immunohistochemical findings have been described with strong emphasis on the diagnostic and therapeutic management of this rare entity.