Management of scalp dermatofibrosarcoma protuberans

Abstract

Background: Dermatofibrosarcoma protuberans is a relatively rare, locally aggressive soft tissue tumor. Less than 5% of these tumors are located on the scalp; however, the recurrence rates for this region are high compared with those of more common locations, such as the trunk and proximal extremities. Standards for the management of dermatofibrosarcoma protuberans in general have been established in the literature, including wide local excision and Mohs micrographic surgery.

Objective: To describe optimum treatment options for primary and recurrent dermatofibrosarcoma protuberans of the scalp based on cases from Roswell Park Cancer Institute (RPCI) and from the literature.

Methods: Five cases from RPCI and 23 cases from the literature were reviewed. Age, sex, history, tumor size and level of invasion, treatment, reconstruction method, and follow-up were compiled from each case.

Results: Twenty-eight cases of scalp dermatofibrosarcoma protuberans were found; 13 were primary, 8 were recurrent, and 3 had persistent positive margins. Fifty-eight percent had classic histologic findings; seven tumors invaded beyond the periosteum. Twenty tumors were treated by surgical excision, whereas eight tumors were treated with Mohs micrographic surgery, requiring between two and four layers. Advanced surgical reconstruction was used in most cases to close the defects. Four patients experienced one tumor recurrence, whereas three other patients had several local recurrences. One patient died of his disease, and one died during surgical treatment of dermatofibrosarcoma protuberans.

Conclusions: Mohs micrographic surgery or a modified version may be the best treatment option for scalp dermatofibrosarcoma protuberans to ensure complete removal of tumor and minimize recurrence. Surgeons should be prepared for advanced reconstruction following tumor removal.