J Cancer 2017; 8(7):1319-1323. doi:10.7150/jca.17988

Short Research Communication

Clinical Features, Pathological Findings and Treatment of Recurrent Dermatofibrosarcoma Protuberans

Yanan Li1, Chuan Wang1, Bo Xiang1, Siyuan Chen2✉, Li Li3, Yi Ji1✉

1. Division of Oncology, Department of Pediatric Surgery, West China Hospital of Sichuan University, Chengdu, 610041, China;
2. Pediatric Intensive Care Unit, Department of Critical Care Medicine, West China Hospital of Sichuan University, Chengdu, 610041, China;
3. Laboratory of Pathology, West China Hospital of Sichuan University, Chengdu, 610041, China.

Abstract

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.

Keywords: Dermatofibrosarcoma protuberans, Recurrence, Clinical features, Mohs micrographic surgery.

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How to cite this article:
Li Y, Wang C, Xiang B, Chen S, Li L, Ji Y. Clinical Features, Pathological Findings and Treatment of Recurrent Dermatofibrosarcoma Protuberans. J Cancer 2017; 8(7):1319-1323. doi:10.7150/jca.17988. Available from http://www.jcancer.org/v08p1319.htm