Dermatofibrosarcome protubérant de l’enfant

Abstract

Dermatofibrosarcoma protuberans (DFSP) is a relatively rare skin neoplasm. Usually affecting adults, the incidence in children is even less frequent. Through a report of three pediatric cases of DFSP, we describe the particularities of this tumor in children. Three boys aged 8, 9, and 15 years presented with a firm nodular skin lesion of the trunk, varying in size from 1 to 5 cm. No previous trauma event had occurred. Diagnosis was confirmed in all cases by immunohistological study. Surgical excision was performed in all cases. No recurrence was evident during the follow-up period of, respectively, 15, 36, and 49 months. The DFSP is an infiltrative tumor of intermediate malignancy, with a limited potential for metastasis (<5%) but a high rate of local recurrence (≥ 50%). The incidence in children is even less frequent. In children, its seemingly benign clinical appearance may explain delays in diagnosis; the majority of lesions affect the extremities, suggesting a potential role-played by injury. In our observations, however, as in adults, the trunk was the site of occurrence. Despite the uncertain pathogenesis of this tumor, the finding of certain characteristic histopathological features helps establish an accurate diagnosis. As in adults, surgical treatment with large surgical margins remains the best practice for children with DFSP, directly affecting the prognosis.