THE MOLECULAR-GENETICS OF VESTIBULAR SCHWANNOMA

Vestibular schwannoma occurs both as a sporadic tumour and in the domi nantly inherited familial cancer syndrome neurofibromatosis type 2 (NF 2). The gene for NF2 has recently been isolated on chromosome 22, and the demonstration of inactivating germline mutations in NF2 patients a nd NF2 associated tumours suggests that it acts as a tumour suppressor . The results of recent research in Cambridge suggest that somatic mut ations of the NF2 tumour suppressor gene are a critical step in the pa thogenesis of both familial and indeed non-familial unilateral sporadi c vestibular schwannoma and that the mechanism of tumourigenesis compl ies with the 'two-hit' model. This paper represents a brief review of the current status of molecular biology in relation to vestibular schw annoma in particular and is discussed in relation to the molecular pat hology of skull base tumours as a whole.