Long-term follow-up of solitary intracerebral juvenile xanthogranuloma. Case report and review of the literature.

Juvenile xanthogranuloma is a benign, non-Langerhans-cell histiocytic infiltrate that typically presents as a solitary cutaneous lesion in childhood. There are reports of extracutanous involvement, including tumors in the central nervous system. A solitary, intraparenchymal tumor without skin manifestations is a rare event, with only 3 prior cases reported in the literature. Cerebral lesions have been associated with multifocal or systemic forms of the disease, with an occasionally fulminate clinical course. Considering the rarity of this tumor, it is unclear whether patients need adjuvant therapy after excision of a solitary intraparenchymal tumor. Previous reports suggested that complete excision of the lesion was curative; however long-term follow-up was not provided. This report illustrates a case of surgical excision of a solitary juvenile xanthogranuloma in an 8-year-old male with a 3-year follow-up period. Copyright 2008 S. Karger AG, Basel.