DOI: 10.36347/sasjm.2023.v09i04.032

Pages: 373-377

Introduction: Darier and Ferrant dermatofibrosarcoma is a rare malignant cutaneous mesenchymal tumour, representing less than 0.1% of malignant tumours and less than 5% of soft tissue sarcomas in adults. The aim of this study was to describe the evolutionary profile of cases of dermato fibrosarcoma of Darier et Ferrant reported in the dermato-venereology department of the National Centre for Disease Control (CNAM). Patients and methods: This was a descriptive cross-sectional study conducted in the dermatology department of the National Centre for Disease Control Support between 1991 and 2016, a period of 25 years. Was included, all patient with a histologically confirmed dermatofibrosarcoma notified in the registers of the CNAM histology laboratory or in the patient's medical file during the study period. Results: In total we collected 23 cases out of 5520 biopsied patients, i.e. a proportion of 0.41%. Males represented 60.8% with an average age of 40 years. The lesions were simple nodular in 12 of the 23 patients (52.2%), multi-nodular in 7 of the 23 patients (30.4%), nodulo-ulcerous and in patches in 4 of the 23 patients (17.4%). The lesions were located on the trunk in 60.8% of cases (image 1), on the head in 21.7% of cases and on the limbs in 17.5% of cases. The mean size of the lesions was 10×8cm with extremes of 3cm×3cm to 25cm×20cm. Management was exclusively surgical. Conclusion: DFSP is a rare tumour with a slow evolution, characterised by its rare metastasis but above all by its strong tendency to recurrence. The diagnosis is often evoked clinically and confirmed by histological study. The treatment of DFSP is surgical based on a large and deep lesion removal. Clinical monitoring allows early detection of recurrence, which is frequent in this disease.