Background: Dermatofibrosarcoma protuberans is an uncommon, locally aggressive but rarely metastasizing tumor of the deep dermis and subcutaneous tissue. It may occur at almost any site but is most common in the trunk and extremities. Dermatofibrosarcoma protuberans of the breast has been rarely reported.
Case presentation: A 49-year-old woman without a significant medical history presented with a right breast mass that had been present for more than 10 years. Recently, progressive enlargement of the mass was noted. Physical examination showed a firm, well-defined, non-tender, mobile, erythematous 2 x 2 cm mass in the upper outer part of the right breast. Breast sonography showed a 2.3 x 1 cm slightly heteroechoic nodule with uneven anterior contours located in the dermal layer, highly suggestive of a dermal lesion. Wide excision of the mass was performed. Microscopically, the hypercellular tumor was composed of relatively monomorphic spindle cells forming a storiform pattern around the sweat glands of the deep dermis and the subcutaneous fat vacuoles. The tumor cells showed strong immunoreactivity for CD34. The final histological diagnosis was dermatofibrosarcoma protuberans.
Conclusions: Dermatofibrosarcoma protuberans of the breast, though uncommon, does exist. With the awareness of this entity can a prompt diagnosis be made and the disease be properly managed.