Le dermato fibrosarcome de DARIER et FERRAND : expérience du centre régional d’oncologie Hassan II Oujda (A propos de 10 cas)

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AuteurBoujtat K
DisciplineCentre Hospitalier Universitaire Mohammed VI d'Oujda
EncadrantEl harroudi T

The dermatofibrosarcoma protuberans is considered a real anatomical and clinical entity by Darier and Ferrand since 1924. It’s a soft tissue tumor ,included in fibroblastic/myofibroblastic tumors, categorized as a rarely metastasizing (intermediate) tumor. It is a rare tumor, distinguished by a slow development, difficulties of diagnostic, local aggressiveness, high recurrence and the rarity of metastases. We report the experience of the Regional Centre of Oncology Hassan II of Oujda by a retrospective study of 10 cases over 6 years from 2008 to 2013. We will discuss the epidemiological, clinical, histological, therapeutic and development features of this tumor. A review of the world literature, comparing the results to ours too was performed. The average age was 45,1 years with an age range of 29 to 71 years. An equal sex distribution (sex-ratio 1). The lesions were multinodular in 6 cases, nodular in 4 cases. The tumors were ulcerated in 2 cases and painful in 1 case. It occurs most commonly on the trunk (40 %) and proximal extremities (30 %), 10 % on the head and neck, we found 2 distal Localizations. The tumors measured 20 cm in greatest diameter and the average size is 5,7 cm. Histologically, each of the tumours was composed of spindle-shaped cells arranged into irregular, interwoven fascicles, resulting in a storiform pattern. Immunohistochemical stains have been used in 5 cases. In all cases tumor cells were reactive to anti-CD34 antibody. The treatment consisted in a wide and deep surgical excision with sacrifice of a healthy anatomical barrier. 1 patient necessitates amputation for tumor infiltrating the bone. In 1case, the surgical margins were not clear. Radiotherapy was delivered to 1 patient with multiple recurrent DFSP. After a median outcome of 33,7 months ranging from 6 to 60 months, we did not note any recurrence or metastasis.