Dermatofibrosarcoma protuberans.

BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is a locally aggressive soft tissue sarcoma in which recurrences are common. It usually affects middle-aged individuals with the most common location being the trunk. Sex distribution varies among published series. Mohs surgery is the treatment of choice. Immunostaining for CD34 facilitates the diagnosis of DFSP and aids in indicating the surgical margins of the tumor; however, the sensitivity of this marker is variable.

MATERIALS AND METHODS: This is a retrospective review of 16 cases of DFSP treated with Mohs surgery at Duke University Medical Center between 1981 and 1994. Clinical and histologic features are analyzed. Immunohistochemical stains for CD34 were performed in one case.

RESULTS: Twelve of the 16 patients were women with four younger than 20 years. The most common location was the trunk. No recurrences following Mohs surgery have been documented. The average number of stages during Mohs surgery was 3.3. Surgical wounds were repaired by complex layered closure in 11 cases. Stains for CD34 were negative on the initial biopsy, but were positive on frozen sections.

CONCLUSIONS: Both sexes and all age groups are affected by DFSP. Mohs surgery is the treatment of choice and offers a significant improvement in cure rates with the cosmetic advantage of smaller postoperative wounds. Immunostains for CD34 appear to be an important adjunct to facilitate tumor removal, but the variable expression of CD34 antigen by DFSP tumors can lead to variable staining patterns.