Dermatofibrosarcoma protuberans. A single center retrospective experience

Abstract

AIM: Dermatofibrosarcoma protuberans (DFSP) is a rare, slowly growing, painless mesenchimal tumor particularly originating from cutaneous and subcutaneous tissues. This neoplasy mostly presents as protrude indurated plaque with brownreddish color or same color of the skin. DFSP has a high rate of recurrence but a low rate of metastasis.

METHODS: We present a retrospective study of 23 patients who were diagnosed with DFSP and operated at our institution. We examined the clinicopathological parameters with clinical outcome and the follow-up.

RESULTS: We retrospectively analysed the data of 23 patients operated for DFSP in Ankara University Medical School Department of Surgical Oncology between 2006 and 2017. Out of these 23 patients, 14 of them were male and 9 of them were female. Dermatofibrosarcoma protuberans has been detected within body in 13 patients, extremities in 7 patients, chest in 2 patients, neck in one patient as well. 6 patients were opereted one times and 17 patients were operated twice due to getting tumor free margins. One patient devaloped local recurrence and reexcision was performed. All patients are still alive and follow up period varied from 12 up to 144 months with a median of 54.2.

CONCLUSION: In conclusion, DFSP is an uncommon, low-grade sarcoma of dermal fibroblast origin with a high local recurrence rate. Diagnosis is established by histology and immunohistochemistry. The greatest clinical challenge in the management of DFSP is achieving local control. Surgical excision is the treatment of choice.