The molecular biology and novel treatments of vestibular schwannomas

作者: Brendan Fong , Garni Barkhoudarian , Patrick Pezeshkian , Andrew T. Parsa , Quinton Gopen

DOI: 10.3171/2011.6.JNS11131

关键词:

摘要: Vestibular schwannomas are histopathologically benign tumors arising from the Schwann cell sheath surrounding vestibular branch of cranial nerve VIII and related to NF2 gene its product merlin. Merlin acts as a tumor suppressor mediator contact inhibition. Thus, deficiencies in both genes lead schwannoma development. Recently, there have been major advances our knowledge molecular biology well development novel therapies for treatment. In this article authors comprehensively review recent characterization modern treatments schwannoma. For instance, merlin is involved with number receptors including CD44 receptor, EGFR, signaling pathways, such Ras/raf pathway canonical Wnt pathway. was also shown interact nucleus E3 ubiquitin ligase CRL4(DCAF1). A greater understanding mechanisms behind tumorigenesis has begun yield therapies. Some that Avastin induces regression progressive by over 40% improves hearing. An inhibitor VEGF synthesis, PTC299, currently Phase II trials potential agent treat Furthermore, vitro studies trastuzumab (an ERBB2 inhibitor) reduces proliferation. With further research it may be possible significantly reduce morbidity mortality rates decreasing burden, volume, hearing loss, deficits seen schwannomas.

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