Clinical Features, Pathological Findings and Treatment of Recurrent Dermatofibrosarcoma Protuberans

Dermatofibrosarcoma protuberans (DESP) is a relatively rare and low-grade tumor of the skin and soft tissue. We review the clinical features, pathological findings and management of recurrent DESP with the aim to improve our understanding of this rare tumor. Fifty-seven patients were diagnosed with recurrent DFSP. The mean age at the presentation of DFSP was 30.9 years. The mean age at diagnosis of DFSP was 40.2 years. Chest wall was the dominant location. The histopathological diagnosis was ordinary DFSP (O-DFSP) in 46 cases and fibrosarcomatous DFSP (FS-DFSP) in 11 cases. No differences were noted between FS-DFSP and conventional DFSP in age at presentation and diagnosis, tumor size, as well as CD34 and SMA expression (P > 0.05). However, the time course to recurrence in FS-DFSP group was less than that in O-DFSP group (P = 0.038). All of the patients were treated with Mohs micrographic surgery (MMS). On follow-up, none of the patients had tumor recurrence. Our data demonstrated that the clinical features of recurrent DFSP are non-specific and variable. Our current practice suggests that MMS is very useful treatment choice for recurrent DFSP.