Dermatofibrosarcoma protuberans (DFSP) is a rare soft tissue tumour of the dermis. Diagnosis may be very difficult from both a clinical and histopathological standpoint. DFSP has a peak incidence in patients between age 10 and 50 years old, occurs with relatively equal frequency in males and females and has a predilection to occur on the trunk or proximal limbs. The tumour has a very low metastatic potential but can be very locally aggressive, requiring wide surgical resection. The possible variants of DFSP may obscure diagnosis especially in the case of fibrohistiocytic lesions such as dermatofibroma, fibromatosis and malignant fibrous histiocytoma. Immunohistological testing, while not completely specific, shows promise in improving diagnostic accuracy. Wide local excision continues to be the mainstay of treatment, although Mohs’ micrographic surgery may ultimately provide superior cure rates.
