An autosomal dominant disorder characterized by a high incidence of bilateral acoustic neuromas as well as schwannomas (NEURILEMMOMA) of other cranial and peripheral nerves, and other benign intracranial tumors including meningiomas, ependymomas, spinal neurofibromas, and gliomas. The disease has been linked to mutations of the NF2 gene (GENES, NEUROFIBROMATOSIS 2) on chromosome 22 (22q12) and usually presents clinically in the first or second decade of life.

Annotation

do not confuse with NEUROFIBROMATOSIS 1; coord IM with precoord/neopl term (IM) if relevant

DUI

D016518 MeSH Browser

CUI

M0025211

Previous indexing

Neurilemmoma (1966-1976); Neurofibromatosis 1 (1966-1991); Neuroma, Acoustic (1978-1991)

History note

1992; NEUROFIBROMATOSIS, CENTRAL, NF2 use NEUROFIBROMATOSIS, CENTRAL, NF-2 use NEUROMA, ACOUSTIC 1989-1991; NEUROMA, ACOUSTIC, BILATERAL use NEUROMA, ACOUSTIC 1989-91

Public note

1992; NEUROFIBROMATOSIS, CENTRAL, NF2 see NEUROFIBROMATOSIS, CENTRAL, NF-2 see NEUROMA, ACOUSTIC 1989-1991; NEUROMA, ACOUSTIC, BILATERAL see NEUROMA, ACOUSTIC 1989-1991