Un sarcome cutané congénital rare : la tumeur fibrohistiocytaire plexiforme

We report the unusual case of a three-year-old girl which presented since birth a pigmented tumor of the left side 0.5 cm in diameter. Surgical removal was decided given the hypothesis of a congenital naevo-cellular naevus. The histological study ended with the diagnosis of plexiform fibrohistiocytic tumor (PFHT). Two other congenital PFHT have been reported until now. PFHT is a rare mesenchymal neoplasm of intermediate malignancy, first reported by Enzinger and Zhang in 1988 which may be difficult to diagnose, because of its low frequency, particulary in congenital cases. It is important to distinguish it from others childhood cutaneous tumors (particulary plexiform neurofibroma, cellular neurothekeoma, infantile myofibromatosis, and fibrous hamartoma). The tumor has a high local recurrence rate and complete surgical resection of the tumor, with wider margins, is required.