Dermatofibrosarcoma protuberans, fibrosarcomatous variant: A rare tumor in children

Abstract

Fibrosarcomatous transformation of dermatofibrosarcoma protuberans is associated with a significantly worse prognosis in adults, but is a very rare feature in the pediatric population. Here, we report a case that occurred in a child. The diagnosis of fibrosarcomatous transformation of dermatofibrosarcoma protuberans was confirmed by a histopathological assessment and fluorescence in situ hybridization. A comparison with eleven other patients reported in the literature revealed that the local recurrence and mortality rates in children are similar to those observed in adults.

Keywords: COL1A1-PDGFB; children; dermatofibrosarcoma protuberans; fibrosarcoma; malignant transformation.