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The molecular genetics of vestibular schwannoma

Published online by Cambridge University Press:  29 June 2007

David A. Moffat  and
Richard M. Irving
David A. Moffat*
Affiliation:
Department of Otoneurological and Skull Base Surgery. Addenbrooke's Hospital, Cambridge.
Richard M. Irving
Affiliation:
Department of Otoneurological and Skull Base Surgery. Addenbrooke's Hospital, Cambridge.
*
Address for correspondence: Mr D. A. Moffat, B.Sc, F.R.C.S., Department of Otolaryngology, Clinic 10, Addenbrooke's Hospital. Hills Road,Cambridge CB2 2QQ. Fax: 01223 217559
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Abstract

Vestibular schwannoma occurs both as a sporadic tumour and in the dominantly inherited familial cancer syndrome neurofibromatosis type 2 (NF2). The gene for NF2 has recently beenisolated on chromosome 22, and the demonstration of inactivating germline mutations in NF2 patients and NF2 associated tumours suggests that it acts as a tumour suppressor. The results of recent research in Cambridge suggest that somatic mutations of the NF2 tumour suppressor gene are a critical step in the pathogenesis of both familial and indeed non-familial unilateral sporadic vestibular schwannoma and that the mechanism of tumourigenesis complies with the ‘two-hit’ model. This paper represents a brief review of the current status of molecular biology in relation to vestibular schwannoma in particular and is discussed in relation to the molecular pathology of skull base tumours as a whole.

Keywords

Vestibular schwannomageneticsbiochemical
Type
Review Articles
Information
The Journal of Laryngology & Otology , Volume 109 , Issue 5 , May 1995 , pp. 381 - 384
Copyright
Copyright © JLO (1984) Limited 1995

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