Darier and Ferrand's Dermatofibrosarcoma with Craniofacial Location: A Rare Entity

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Introdcuction: Darier and Ferrand's dermatofibrosarcoma is a rare but not exceptional malignant mesenchymentous skin tumor. It represents 0.1% of malignant skin tumors. First described by Jean Darier and Marcel Ferrant in 1924, it is a slow-growing tumor, currently considered to be a true low-grade malignancy fibrosarcoma. The authors report in this work the clinical and evolutionary aspects of three cases of Darier and Ferrand dermatofibrosarcoma located at the cephalic end. Observations: These were 3 cases of Darier and Ferrand dermatofibrosarcoma encountered between 2010 and 2016. The subjects were female. The ages ranged from 30 to 44 years old. The diagnostic delay varied between 6 and 10 years. The general condition was altered in two cases. The localization was facial in two cases and the scalp in one case. The size of the tumors varied between 15 and 20 cm. All patients underwent maxillofacial computed tomography (CT) and laboratory workup. The clinically suggested diagnosis was confirmed by histological examination. One patient underwent surgical excision with a 3 cm margin. Covering the loss of substance appealed to thin skin grafting. The evolution was favorable after a 7-year follow-up. In the other two cases, the evolution was marked by death before surgical management. Conclusion: DFS is a rare malignant skin tumor with slow local progression that can often be life-threatening. Early diagnosis ensures efficient treatment.

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