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The neurofibromatoses are a diverse group of genetic conditions that share a predisposition to the development of tumors of the nerve sheath. Schwannomatosis is a recently recognized third major form of neurofibromatosis (NF) that causes multiple schwannomas without vestibular tumors diagnostic of NF2. Patients with schwannomatosis represent 2.4 to 5% of(More)
Neurofibromatosis type 2 (NF2) is an autosomal dominant inherited tumor predisposition syndrome caused by mutations in the NF2 gene on chromosome 22. Affected individuals develop schwannomas typically involving both vestibular nerves leading to hearing loss and eventual deafness. Rehabilitation with brainstem implants and in some cases cochlear implants is(More)
A consensus conference on neurofibromatosis 2 (NF2) was held in 2002 at the request of the United Kingdom (UK) Neurofibromatosis Association, with particular emphasis on vestibular schwannoma (VS) surgery. NF2 patients should be managed at specialty treatment centres, whose staff has extensive experience with the disease. All NF2 patients and their families(More)
BACKGROUND Some 4%-5% of those who develop vestibular schwannomas have neurofibromatosis type 2 (NF2). Although about 10% of these patients present initially with a unilateral vestibular schwannoma, the risk for a patient with a truly sporadic vestibular schwannoma developing contralateral disease is unknown. METHODS A United Kingdom survey of 296(More)
Neurofibromatosis 2 (NF2) is a rare (1:40 000) autosomal dominant disease that is caused by mutations of the NF2 tumour suppressor gene. NF2 is characterized by benign nervous system tumours such as vestibular schwannomas (VSs), intracranial meningiomas and spinal tumours. Kondziolka et al (1998) reported the outcomes of radiosurgery for unilateral sporadic(More)
OBJECTIVE To determine the specificity of the current clinical diagnostic criteria for neurofibromatosis type 2 (NF2) relative to the requirement for unilateral vestibular schwannoma (VS) and at least 2 other NF2-related tumors. METHODS We interrogated our Manchester NF2 database, which contained 205 individuals meeting NF2 criteria who initially(More)
Neurofibromatosis type 2 (NF2; MIM # 101000) is an autosomal dominant disorder characterized by the development of vestibular schwannomas (VSs); schwannomas of other cranial, spinal, and cutaneous nerves; cranial and spinal meningiomas or other central nervous system tumors (eg, ependymomas and astrocytomas) or both. Additional features include eye (eg,(More)
BACKGROUND The current study examines the hypothesis that tumor growth rate in sporadic vestibular schwannoma could be in part predicted by data available at diagnosis, such as tumor volume, patient age, laterality, and sex. METHODS Tumor volumes and growth rates were calculated from serial magnetic resonance imaging data in a cohort of 50 patients (26(More)
OBJECTIVE To delineate the clinical phenotype, molecular basis, and implications for screening in patients and families with multiple schwannomas not generally involving the cranium. METHODS As part of a United Kingdom clinical and genetic study of type 2 neurofibromatosis (NF2) patients and families with multiple schwannomas who do not fulfil diagnostic(More)
Diagnostic criteria for schwannomatosis have been proposed in a recent consensus statement. These criteria permit schwannomatosis to be distinguished from neurofibromatosis type 2 (NF2) in most patients, but there is some clinical overlap between the two diseases. In this study, the authors use data from the population-based United Kingdom NF2 Registry to(More)