chapter  59
Neurofibromatosis type 2
Pages 14

Neurofibromatosis 2 is an autosomal dominant disorder, caused by a germline mutation and inactivation of the NF2 tumor suppressor gene. The disease is characterized clinically by the development of multiple tumors including bilateral vestibular schwannomas, spinal and cranial schwannomas, meningiomas and ependymomas, as well as other, nonsymptomatic, dysplastic lesions.