Abstract
Bilateral vestibular schwannomata and meningiomata are the tumours most commonly associated with neurofibromatosis type II (NF2). These tumours may also be seen in patients with schwannomatosis and familial meningioma, but these phenotypes are usually easy to distinguish. The main diagnostic challenge when managing these tumours is distinguishing between sporadic disease which carries low risk of subsequent tumours or NF2 with its associated morbidities and reduced life expectancy. This chapter outlines some of the diagnostic and management considerations along with associated evidence.
Keywords
- Vestibular schwannoma
- Meningioma
- Neurofibromatosis type II
- NF2
- Schwannomatosis
This is a preview of subscription content, access via your institution.
Buying options
Tax calculation will be finalised at checkout
Purchases are for personal use only
Learn about institutional subscriptionsReferences
Aavikko M, Li SP, Saarinen S, Alhopuro P, Kaasinen E, Morgunova E, Li Y, Vesanen K, Smith MJ, Evans DG, Pöyhönen M, Kiuru A, Auvinen A, Aaltonen LA, Taipale J, Vahteristo P (2012) Loss of SUFU function in familial multiple meningioma. Am J Hum Genet 91(3):520–526
Asthagiri AR1, Parry DM, Butman JA, Kim HJ, Tsilou ET, Zhuang Z, Lonser RR (2009) Neurofibromatosis type 2. Lancet 373(9679):1974–1986
Bacci C1, Sestini R, Provenzano A, Paganini I, Mancini I, Porfirio B, Vivarelli R, Genuardi M, Papi L (2010) Schwannomatosis associated with multiple meningiomas due to a familial SMARCB1 mutation. Neurogenetics 11(1):73–80
Baser ME, Friedman JM, Wallace AJ, Ramsden RT, Joe H, Evans DG (2002) Evaluation of clinical diagnostic criteria for neurofibromatosis 2. Neurology 59(11):1759–1765
Baser ME, Kuramoto L, Joe H, Friedman JM, Wallace AJ, Gillespie JE, Ramsden RT, Evans DG (2004) Genotype-phenotype correlations for nervous system tumors in neurofibromatosis 2: a population-based study. Am J Hum Genet 75:231–239
Carlson ML, Tveiten OV, Driscoll CL, Goplen FK, Neff BA, Pollock BE, Tombers NM, Castner ML, Finnkirk MK, Myrseth E, Pedersen PH, Lund-Johansen M, Link MJ (2015) Long-term quality of life in patients with vestibular schwannoma: an international multicenter cross-sectional study comparing microsurgery, stereotactic radiosurgery, observation, and nontumor controls. J Neurosurg 122(4):833–842
Dirks MS, Butman JA, Kim HJ, Wu T, Morgan K, Tran AP, Lonser RR, Asthagiri AR (2012) Long-term natural history of neurofibromatosis Type 2-associated intracranial tumors. J Neurosurg 117(1):109–117
Evans DG, Huson SM, Donnai D, Neary W, Blair V, Newton V, Harris R (1992) A clinical study of type 2 neurofibromatosis. Q J Med 84:603–618
Ferner RE, Shaw A, Evans DG, McAleer D, Halliday D, Parry A, Raymond FL, Durie-Gair J, Hanemann CO, Hornigold R, Axon P, Golding JF (2014) Longitudinal evaluation of quality of life in 288 patients with neurofibromatosis 2. J Neurol 261(5):963–969
Karajannis MA, Legault G, Hagiwara M, Giancotti FG, Filatov A, Derman A, Hochman T, Goldberg JD, Vega E, Wisoff JH, Golfinos JG, Merkelson A, Roland JT, Allen JC (2014) Phase II study of everolimus in children and adults with neurofibromatosis type 2 and progressive vestibular schwannomas. Neuro Oncol 16(2):292–297
Karajannis MA, Ferner RE (2015) Neurofibromatosis-related tumors: emerging biology and therapies. Curr Opin Pediatr 27(1):26–33
Larjavaara S, Haapasalo H, Sankila R, Helén P, Auvinen A (2008) Is the incidence of meningiomas underestimated? A regional survey. Br J Cancer 99(1):182–184
Lin D, Hegarty JL, Fischbein NJ, Jackler RK (2005) The prevalence of “incidental” acoustic neuroma. Arch Otolaryngol Head Neck Surg 131(3):241–244
Mallory GW, Pollock BE, Foote RL, Carlson ML, Driscoll CL, Link MJ (2014) Stereotactic radiosurgery for neurofibromatosis 2-associated vestibular schwannomas: toward dose optimization for tumor control and functional outcomes. Neurosurgery 74(3):292–300
Moffat DA, Lloyd SK, Macfarlane R, Mannion R, King A, Rutherford S, Axon PR, Donnelly N, Freeman S, Tysome JR, Evans DG, Ramsden RT (2013) Outcome of translabyrinthine surgery for vestibular schwannoma in neurofibromatosis type 2. Br J Neurosurg 27(4):446–453
Nunes FP, Merker VL, Jennings D, Caruso PA, di Tomaso E, Muzikansky A, Barker FG 2nd, Stemmer-Rachamimov A, Plotkin SR (2013) Bevacizumab treatment for meningiomas in NF2: a retrospective analysis of 15 patients. PLoS One 8(3):e59941
Plotkin SR, Stemmer-Rachamimov AO, Barker FG 2nd, Halpin C, Padera TP, Tyrrell A, Sorensen AG, Jain RK, di Tomaso E (2009) Hearing improvement after bevacizumab in patients with neurofibromatosis type 2. N Engl J Med 361(4):358–367
Plotkin SR, O’Donnell CC, Curry WT, Bove CM, MacCollin M, Nunes FP (2011) Spinal ependymomas in neurofibromatosis Type 2: a retrospective analysis of 55 patients. J Neurosurg Spine 14(4):543–547
Slusarz KM, Merker VL, Muzikansky A, Francis SA, Plotkin SR (2014) Long-term toxicity of bevacizumab therapy in neurofibromatosis 2 patients. Cancer Chemother Pharmacol 73(6):1197–1204
Smith MJ, Higgs JE, Bowers NL, Halliday D, Patterson J, Gillespie J, Huson SM, Freeman SR, Lloyd S, Rutherford SA, King AT, Wallace AJ, Ramsden RT, Evans DG (2011) Cranial meningiomas in 411 NF2 patients with proven gene mutations: clear positional effect of mutations, but absence of female severity effect on age at onset. J Med Genet 48:261–265
Smith MJ, O’Sullivan J, Bhaskar SS, Hadfield KD, Poke G, Caird J, Sharif S, Eccles D, Fitzpatrick D, Rawluk D, du Plessis D, Newman WG, Evans DG (2013) Loss-of-function mutations in SMARCE1 cause an inherited disorder of multiple spinal meningiomas. Nature Genet 45:295–298
Stangerup SE, Tos M, Thomsen J, Caye-Thomasen P (2010) True incidence of vestibular schwannoma? Neurosurgery 67(5):1335–1340
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2016 Springer International Publishing Switzerland
About this chapter
Cite this chapter
Shaw, A. (2016). Diagnosis and Management of Hereditary Meningioma and Vestibular Schwannoma. In: Pichert, G., Jacobs, C. (eds) Rare Hereditary Cancers. Recent Results in Cancer Research, vol 205. Springer, Cham. https://doi.org/10.1007/978-3-319-29998-3_2
Download citation
DOI: https://doi.org/10.1007/978-3-319-29998-3_2
Published:
Publisher Name: Springer, Cham
Print ISBN: 978-3-319-29996-9
Online ISBN: 978-3-319-29998-3
eBook Packages: MedicineMedicine (R0)