Abstract

Marouane Habla*, Jaafari Ahmed Zakaria, Benkhakdoun Mohamed, Benyoussef Jihane, Zahiri Saad, El Youssefi Ahlam, El Harti Amine and Mounia Diouri

ABSTRACT

Darier and Ferrand dermatofibrosarcoma (DFS) is a fibrous skin tumor of intermediate malignancy. It is a rare but not exceptional tumor, infiltrating the dermis and hypodermis representing 0.1% to 1% of malignant skin tumors. Characterized by a very polymorphic clinical appearance, slow growth, a very high risk of local recurrence, a low metastatic potential. The treatment is surgical and well codified (Chemotherapy: Imatinib). Its term “recurrent” is linked to insufficient excisions, hence the importance of well-conducted primary surgery and regular clinical and radiological monitoring. We present a retrospective study of 18 cases of DFS diagnosed over a period of 4 years (2020 to 2024) (6 cases de novo - 12 cases of recurrence) and compare it to literature data. This study makes it possible to establish, in addition to the anatomopathological and immunohistochemical characteristics, an epidemiological, clinical and progressive study of this sarcoma. the average age of our patients is 42 years old with a male predominance. The trunk is the preferred location affected in 70% of cases. The tumor size reached 18 cm and measured on average 6.2 cm. The diagnosis was suggested by standard histological examination and confirmed by the intense and diffuse expression of CD34. The evolution was marked by the transformation into a high-grade pleomorphic sarcoma in a case operated initially outside our structure and by a case of local recurrence. Our results are classic and comparable to other series in the literature. Histological examination is essential for diagnosis. Wide surgical excision is the standard treatment. The prognosis is conditioned by mainly local malignancy and a high potential for recurrence. The frankly malignant sarcomatous transformation is exceptional.