Molecular studies of vestibular schwannomas: a review

Abstract

To summarize advances in understanding the molecular biology of vestibular schwannomas over the past year. The role of the neurofibromatosis type 2 protein, denoted as merlin or schwannomin, in embryonic development, cellular adherence, and in cell proliferation has become better elucidated in the past year. Likewise, the role of merlin in Schwann cell-axon interaction has been studied. Additionally, two comprehensive analyses of the spectrum of human neurofibromatosis type 2 mutations have been compiled which make up a valuable resource in understanding critical regions of the neurofibromatosis type 2 gene. Neurofibromatosis type 2 screening guidelines for young patients with solitary vestibular schwannomas have been published. The role of electromagnetic radiation via cellular and portable telephones as a predisposing factor to vestibular schwannoma formation has also been the topic of several studies. Based on increased knowledge of the pathways in which merlin functions and the available transgenic and xenograft mouse models, preliminary data regarding directed pharmacotherapy are also summarized. With increased knowledge of the pathologic mechanisms and interacting proteins associated with merlin, the research community is poised to begin trials of targeted interventions in vitro and in the current mouse models.