NEURILEMMOMATOSIS, NF2, AND JUVENILE XANTHOGRANULOMA

The gene locus for neurilemmomatosis has been reported to Lie within t he neurofibromatosis type 2 gene region, suggesting that these 2 disea ses may be identical. We describe an adolescent girl with multiple neu rilemmomas and juvenile xanthogranulomas, who was found to have bilate ral acoustic neuromas and multiple central nervous tissue tumors as se en in patients with a diagnosis of neurofibromatosis type 2. This case and recent genetic studies suggest that neurilemmomatosis and neurofi bromatosis type 2 are the same disease.