[Dermatofibrosarcoma in children. Report of 7 Tunisian cases]

Dermatofibrosarcoma (DFS) protuberans is a rare mesenchyma tumor in childhood characterised by slow growth. We report the result of a retrospective study of 7 childhood (4 males) cases of through a fifteen year period. The mean age was 13 years. 4 Tumors were (DFS) located on the limbs: 2 on the trunk: and on the scalp. Clinical types were multinodular and infiltrated in 3 cases, nodular in two cases and ulcerated with budding also in two cases. The mean delay between diagnostic and the appearance of the tumor was about 24 months for 4 initial tumors. Histological features were typical in all cases. We report 3 recurrences of dermatofibrosarcoma arising few months after surgical excision of an initial tumor, in one case the tumor was diagnosed histocytofibroma. Six patient underwent surgical excision with wide margins, and in one case the margins were considered borderline. No recurrences were reported, the follow-up was between 17 months to 9 years. It's a rare tumor in childhood, 160 cases were reported. The clinical and evolutive mode were entirely comparable to the adult cases. However the initial appearance as benign atrophic or indurated plaque is more frequent in childhood, but this appearance wasn't noted in our series.