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Darier-Ferrand dermatofibrosarcoma protuberans of the face: Case report and literature review
Souheil Jbali1, Rim Braham1, Malek Driss1
1Head and Neck Department, Salah Azaiez Institute of Cancer, Tunis University of Medicine, University Tunis El Manar, Tunis, Tunisia.
Introduction:
Darier Ferrand Dermatofibrosarcoma (DFSP) is a rare skin tumor, characterized by its local aggressivity and high potential of recurrence. It affects mainly young and middle-aged adults. Its location in the head and neck region represents nearly 12.5 % of all DFSP.
Case Description:
We report a rare case of a 68-year-old patient who was treated for a DFSP of the cheek's lower part with reconstruction using a pectoralis major musculocutaneous flap. The postoperative course was uneventful and the aesthetic result was good.
Discussion:
DFSP is a tumor of intermediate malignancy. Its optimal management is based on complete surgical excision requiring various reconstruction procedures for large defects. The treatment of unresectable lesions remains difficult despite the targeted therapies proposed and the improvement of radiotherapy techniques.
Conclusion:
The ideal management of a DFSP is based on surgical excision passing largely in healthy tissues. This situation requires early diagnosis and is the guarantee of disease-free survival without major sequelae in head and neck locations.
