Dermatofibrosarcoma protuberans

Dermatofibrosarcoma protuberans is an uncommon cancer in which tumors arise in the deeper layers of skin. The tumor usually starts as a small, firm patch of skin; it may be purplish, reddish, or flesh-colored. It is commonly found on the torso, usually in the shoulder and chest area. The tumor typically grows slowly but has a tendency to recur after being removed. It rarely spreads to other parts of the body. The cause of DFSP is unknown, but injury to the affected skin may be a predisposing factor. Treatment usually involves surgically removing the tumor. If the tumor is unable to be removed completely, additional therapy may be needed. Regular follow-up is important to monitor for recurrence.^[1][2][3]

Dermatofibrosarcoma protuberans is a slow-growing tumor and does not usually spread to distant parts of the body (metastasize). The overall rate of distant metastasis is estimated to be around 4%, and the rate of metastasis to nearby lymph nodes is 1%.^[4]

Dermatofibrosarcoma protuberans has an average recurrence rate of 15.7% after being removed by standard surgery known as wide local excision (when the entire tumor and some of the surrounding normal tissue are removed).  Most local recurrences occur within 3 years.  Recent studies suggest that a newer surgical technique, Mohs’ micrographic surgery, may help reduce this recurrence rate to approximately 1.3%. ^[4]