Kshettry VR, Hsieh JK, Ostrom QT, Kruchko C, Barnholtz-Sloan JS (2015) Incidence of vestibular schwannomas in the united States. J Neurooncol 124(2):223–228. https://doi.org/10.1007/s11060-015-1827-9

Article  PubMed  Google Scholar 

Plotkin SR, Messiaen L, Legius E et al (2022) Updated diagnostic criteria and nomenclature for neurofibromatosis type 2 and schwannomatosis: an international consensus recommendation. Genet Sci 24(9):1967–1977. https://doi.org/10.1016/j.gim.2022.05.007

Article  CAS  Google Scholar 

Guo S, Zheng X, Chen W et al (2024) From bench to bedside: advancing towards therapeutic treatment of vestibular schwannomas. Neuro-Oncol Adv 6(1):vdae107. https://doi.org/10.1093/noajnl/vdae107

Article  Google Scholar 

Bachir S, Shah S, Shapiro S et al (2021) Neurofibromatosis type 2 (NF2) and the implications for vestibular Schwannoma and meningioma pathogenesis. Int J Mol Sci 22(2):690. https://doi.org/10.3390/ijms22020690

Article  CAS  PubMed  PubMed Central  Google Scholar 

Anaizi AN, DiNapoli VV, Pensak M, Theodosopoulos PV (2016) Small vestibular schwannomas: does surgery remain a viable treatment option? J Neurol Surg Part B Skull Base 77(3):212–218. https://doi.org/10.1055/s-0035-1564591

Article  Google Scholar 

Long J, Zhang Y, Huang X, Ren J, Zhong P, Wang B (2021) A review of drug therapy in vestibular Schwannoma. Drug Des Devel Ther 15:75–85. https://doi.org/10.2147/DDDT.S280069

Article  PubMed  PubMed Central  Google Scholar 

Cumpston EC, Rhodes SD, Yates CW (2023) Advances in targeted therapy for neurofibromatosis type 2 (NF2)-Associated vestibular schwannomas. Curr Oncol Rep 25(5):531–537. https://doi.org/10.1007/s11912-023-01388-3

Article  PubMed  Google Scholar 

Tamura R, Toda M (2022) A critical overview of targeted therapies for vestibular Schwannoma. Int J Mol Sci 23(10):5462. https://doi.org/10.3390/ijms23105462

Article  CAS  PubMed  PubMed Central  Google Scholar 

Lawson McLean AC, Rosahl SK (2017) Growth dynamics of intracranial tumors in patients with neurofibromatosis type 2. World Neurosurg 98:152–161. https://doi.org/10.1016/j.wneu.2016.10.060

Article  PubMed  Google Scholar 

Evans DgR (2009) Neurofibromatosis type 2 (NF2): A clinical and molecular review. Orphanet J Rare Dis 4(1):16. https://doi.org/10.1186/1750-1172-4-16

Article  PubMed  PubMed Central  Google Scholar 

Mautner VF, Baser ME, Thakkar SD, Feigen UM, Friedman JM, Kluwe L (2002) Vestibular Schwannoma growth in patients with neurofibromatosis type 2: a longitudinal study. J Neurosurg 96(2):223–228. https://doi.org/10.3171/jns.2002.96.2.0223

Article  PubMed  Google Scholar 

Gugel I, Grimm F, Liebsch M et al (2019) Impact of surgery on Long-Term results of hearing in neurofibromatosis Type-2 associated vestibular schwannomas. Cancers 11(9):1376. https://doi.org/10.3390/cancers11091376

Article  PubMed  PubMed Central  Google Scholar 

Sri Krishna GS, Pahwa B, Jagdevan A, Singh M, Kale S, Agrawal D (2023) Tumor control and hearing preservation after gamma knife radiosurgery for vestibular schwannomas in neurofibromatosis type 2-A retrospective analysis of 133 tumors. World Neurosurg 171:e820–e827. https://doi.org/10.1016/j.wneu.2022.12.118

Article  PubMed  Google Scholar 

Neff BA, Welling DB, Akhmametyeva E, Chang LS (2006) The molecular biology of vestibular schwannomas: dissecting the pathogenic process at the molecular level. Otol Neurotol Off Publ Am Otol Soc Am Neurotol Soc Eur Acad Otol Neurotol 27(2):197–208. https://doi.org/10.1097/01.mao.0000180484.24242.54

Article  Google Scholar 

Hilton DA, Hanemann CO (2014) Schwannomas and their pathogenesis. Brain Pathol Zurich Switz 24(3):205–220. https://doi.org/10.1111/bpa.12125

Article  Google Scholar 

Petrilli AM, Fernández-Valle C (2015) Role of Merlin/NF2 inactivation in tumor biology. Oncogene 35(5):537–548. https://doi.org/10.1038/onc.2015.125

Article  CAS  PubMed  PubMed Central  Google Scholar 

Colciago A, Melfi S, Giannotti G et al (2015) Tumor suppressor Nf2/merlin drives Schwann cell changes following electromagnetic field exposure through Hippo-dependent mechanisms. Cell Death Discovery 1(1). https://doi.org/10.1038/cddiscovery.2015.21

Ahmad Z, Brown CM, Patel AK, Ryan AF, Ongkeko R, Doherty JK (2010) Merlin knockdown in human Schwann cells. Otology Neurotology 31(3):460–466. https://doi.org/10.1097/mao.0b013e3181d2777f

Article  PubMed  PubMed Central  Google Scholar 

Fernandez-Valle C, Tang Y, Ricard J et al (2002) Paxillin binds Schwannomin and regulates its density-dependent localization and effect on cell morphology. Nat Genet 31(4):354–362. https://doi.org/10.1038/ng930

Article  CAS  PubMed  Google Scholar 

Lallemand D, Curto M, Saotome I, Giovannini M, McClatchey AI (2003) NF2 deficiency promotes tumorigenesis and metastasis by destabilizing adherens junctions. Genes Dev 17(9):1090–1100. https://doi.org/10.1101/gad.1054603

Article  CAS  PubMed  PubMed Central  Google Scholar 

Gladden AB, Hebert AM, Schneeberger EE, McClatchey AI (2010) The NF2 tumor suppressor, Merlin, regulates epidermal development through the establishment of a junctional Polarity complex. Dev Cell 19(5):727–739. https://doi.org/10.1016/j.devcel.2010.10.008

Article  CAS  PubMed  PubMed Central  Google Scholar 

Curto M, Cole BK, Lallemand D, Liu CH, McClatchey AI (2007) Contact-dependent Inhibition of EGFR signaling by Nf2/Merlin. J Cell Biol 177(5):893–903. https://doi.org/10.1083/jcb.200703010

Article  CAS  PubMed  PubMed Central  Google Scholar 

Lim JY, Kim H, Jeun SS, Kang SG, Lee KJ (2006) Merlin inhibits growth hormone-regulated Raf-ERKs pathways by binding to Grb2 protein. Biochem Biophys Res Commun 340(4):1151–1157. https://doi.org/10.1016/j.bbrc.2005.12.122

Article  CAS  PubMed  Google Scholar 

Flaiz C, Kaempchen K, Matthies C, Hanemann CO (2007) Actin-rich protrusions and nonlocalized GTPase activation in Merlin-deficient schwannomas. J Neuropathol Exp Neurol 66(7):608–616. https://doi.org/10.1097/nen.0b013e318093e555

Article  CAS  PubMed  Google Scholar 

Nakai Y, Zheng Y, MacCollin M, Ratner N (2006) Temporal control of Rac in Schwann cell-axon interaction is disrupted in NF2-mutant Schwannoma cells. J Neurosci Off J Soc Neurosci 26(13):3390–3395. https://doi.org/10.1523/JNEUROSCI.4865-05.2006

Article  CAS  Google Scholar 

Shaw RJ, Paez JG, Curto M et al (2001) The Nf2 tumor suppressor, merlin, functions in Rac-dependent signaling. Dev Cell 1(1):63–72. https://doi.org/10.1016/s1534-5807(01)00009-0

Article  CAS  PubMed  Google Scholar 

Petrilli AM, Fuse MA, Donnan MS et al (2014) A chemical biology approach identified PI3K as a potential therapeutic target for neurofibromatosis type 2. Am J Transl Res 6(5):471–493

PubMed  PubMed Central  Google Scholar 

Rong R, Tang X, Gutmann DH, Ye K (2004) Neurofibromatosis 2 (NF2) tumor suppressor Merlin inhibits phosphatidylinositol 3-kinase through binding to PIKE-L. Proc Natl Acad Sci U S A 101(52):18200–18205. https://doi.org/10.1073/pnas.0405971102

Article  CAS  PubMed  PubMed Central  Google Scholar 

Li W, Cooper J, Zhou L et al (2014) Merlin/NF2 loss-driven tumorigenesis linked to CRL4(DCAF1)-mediated Inhibition of the Hippo pathway kinases Lats1 and 2 in the nucleus. Cancer Cell 26(1):48–60. https://doi.org/10.1016/j.ccr.2014.05.001

Article  CAS  PubMed  PubMed Central  Google Scholar 

Li W, You L, Cooper J et al (2010) Merlin/NF2 suppresses tumorigenesis by inhibiting the E3 ubiquitin ligase CRL4DCAF1 in the nucleus. Cell 140(4):477–490. https://doi.org/10.1016/j.cell.2010.01.029

Article  CAS