DARIER-FERRAND DERMATOFIBROSARCOMA OF THE FACE: TWO CASES REPORT

Abstract

Darier-Ferrand dermatofibrosarcoma or dermatofibrosarcoma protuberans (DFSP) is a cutaneous mesenchymal tumor of intermediate malignancy. It is a rare but not exceptional tumor, accounting for 0.1% of malignant skin tumors. Histological examination is essential for diagnosis. Wide surgical excision is the standard treatment. We report two cases of Darier-Ferrand dermatofibrosarcoma of the face. A wide local excision is the gold-standard treatment, with negative margins of 3–5 cm from the tumor edge including the skin, the subcutaneous tissue, and the underlying fascia. However, this is a mutilating surgery, especially for cervicofacial lesions which is the case for our two patients For cervico-facial localizations,some authors advise the Mohs micrographic surgery for its low recurrence rates described. Target adjuvant therapy with imatinib has been shown to reduce tumor size and improve surgical resectability and can be proposed as an option for our patients. However, its unavailability and high-cost limit its use in our context. A combination of conservative excision and adjuvant radiotherapy was reported to reduce the rate of local recurrence and should be also considered by practicians. To improve prognosis, early, codified, multidisciplinary management is essential for this pathology.