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Dermatofibrosarcoma protuberans, fibrosarcomatous variant: A rare tumor in children
Matthieu Chicaud1,2,3, Annonciade Frassati-Biaggi1,2, Sophie Kaltenbach2,4,5
1Department of Pathology, Necker-Enfants Malades Hospital, Paris, France.
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.
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