ÜMMÜGÜL ÜYETÜRK, TUBA TASLAMACIOĞLU DUMAN, SELMA ERDOĞAN DÜZCÜ, NADİRE KÜÇÜKÖZTAŞ, FAHRİ YILMAZ
Turkish Journal of Oncology - 2017;32(4):148-152
Dermatofibrosarcoma protuberans (DFSP) is a fibrohistiocytic tumor characterized by protuberant growth pattern of skin and subcutaneous tissue. The annual incidence is 4.2 per million. It is often seen between the ages of 20-50 years. This study aimed to evaluate the characteristics of patients with DFSP. METHODS Patients who visited our clinic were retrospectively evaluated. RESULTS All patients were females and the median age was 42 (29-60) years. The evaluated tumor sites were observed in the body/trunk in one patient, at the hip in two, and at the proximal lower extremity in two. Surgical excision was done in all of them as initial treatment. Re-excision was needed in three patients. One of the patients underwent punch biopsy for diagnosis and surgical removal of the tumor with a negative surgical margin. One of the patients underwent radiotherapy. The median follow-up time was 10 (6-26) months. During the median follow-up, there was no local recurrence or metastasis. CONCLUSION As initial treatment, it is important to surgically remove tumors that had no distant metastasis. Positive or near surgical margins should be resected again. If a negative surgical margin cannot be achieved, radiotherapy should be used as the treatment. Imatinib mesylate is used for treatment in unresectable, recurrent, and/or metastatic tumors.
