Vestibular schwannomas (VSs) are benign nerve sheath tumors that are frequently seen along with other nervous system tumors in patients with Neurofibromatosis type 2 (NF2). Current management options include observation by magnetic resonance imaging (MRI), surgical excision, and stereotactic radiation. However, complications from treatment, such as hearing loss, facial weakness, dizziness, intracranial bleeding, and stroke, remain major concerns. In addition, radiation treatment may cause malignant transformation and/or growth acceleration of the tumors. Thus, development of a medical therapy for the treatment of NF2-associated tumors is urgently needed. Recent studies provide new insight into the role of merlin, the NF2 tumor suppressor gene product that is frequently inactivated in VSs, and the signaling pathways that are deregulated during VS tumorigenesis. Several in vitro and in vivo models for NF2-associated tumors have been developed and are utilities for therapeutic testing. A number of exciting research studies on therapeutic interventions for VS have been initiated and show promising results. Hopefully, some of the drugs or pathway-specific inhibitors will prove effective and become alternatives to the current treatment options.
|Original language||English (US)|
|Title of host publication||Advances in Neurofibromatosis Research|
|Publisher||Nova Science Publishers, Inc.|
|Number of pages||16|
|State||Published - Dec 1 2012|
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