[Dermatofibrosarcoma protuberans in children]. / Le dermatofibrosarcome chez l'enfant.

ABSTRACT

Dermatofibrosarcoma protuberans is a malignant and locally invasive tumor. It generally affects young adults. However, in rare but not exceptional cases, children can suffer from this disease. In the literature, there are only few studies on this pathology occurring in children. The main treatment is surgical excision with large margins. We studied in our series 15 cases of dermatofibrosarcoma in children between 1995 and 2008. The data were retrospectively collected. The aim of our study is to underline the best support for this pathology in children. The mean age at the moment of diagnosis was 13 years old. None of our patients underwent surgery for dermatofibrosarcoma removal before. The diagnosis was confirmed by biopsy and immunology by FISH method. In 60% of the cases, the lesion, which is very polymorphic, was on the trunk. In three cases, a skin trauma was noted in the patient's history. Surgical management consisted in tumor's excision with a 3 cm margin laterally associated with the removal of the first unaffected anatomical layer in depth. Reconstruction was adapted to the defect's width, depth and topography. The mean follow-up was 7.8 years. There were no relapses reported in a 3-year period at least. Complications were two scars dehiscences which needed skin graft for repair and a scarring alopecia reconstructed secondarily by a skin expansion protocol. No functional sequelae were reported in our series. As dermatofibrosarcoma in children presents the same clinical aspect and evolution as in adults, we treated it the same way. This diagnosis should not be ignored in children, and should be made early to give the patient the most effective support.