Giant juvenile xanthogranuloma of the tongue

Juvenile xanthogranulomas (JXGs) are rare, benign, fibrohistiocytic lesions . They usually appear as one or more cutaneous papules on the head, neck, o r trunk in infants. Twelve cases of oral JXGs have been reported, four of w hich involved the tongue. We present a 6-year-old girl with a large tongue mass diagnosed as JXG after an excisional biopsy. Histological and immunohi stochemical staining results are presented. This is the first reported case of a giant oral JXG. A review of the literature on these unusual lesions i s presented, along with discussion of their differential diagnosis and key aspects of the patient's evaluation, management, and pathological diagnosis . (Am J Otolaryngol 1999;20:241-244. Copyright (C) 1999 by W.B. Saunders Co mpany).