Abstract
Multiple schwannomas occur in association with neurofibromatosis type 2 (NF2) and schwannomatosis. NF2 is a dominantly inherited tumor prediction syndrome which is characterized by bilateral vestibular schwannomas. Schwannomatosis is the third major form of neurofibromatosis and involves the development of multiple schwannomas in the absence of vestibular schwannomas. In general, schwannomas associated with NF2 or schwannomatosis grow slowly and do not become malignant, whereas neurofibromas with NF1 have a propensity for malignant transformation. Usually, these tumors can be detected by magnetic resonance imaging and a diagnosis is made using each criterion. The mainstay of the current treatment for multiple schwannomas is the surgical removal of symptomatic tumors. Considering the natural course of schwannomas and the risk of surgery, the mere presence of a tumor is not an indication for its surgical removal. Radiation therapy is a management option in some patients.
This is a preview of subscription content, log in via an institution to check access.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
References
Asthagiri AR, Parry DM, Butman JA, Kim HJ, Tsilou ET, Zhuang Z, Lonser RR (2009) Neurofibromatosis type 2. Lancet 373:1974–1986
Baser ME, Evans DG, Jackler RK, Sujansky E, Rubenstein A (2000) Neurofibromatosis 2, radiosurgery and malignant nervous system tumours. Br J Cancer 82:998
Beaman FD, Kransdorf MJ, Menke DM (2004) Schwannoma: radiologic-pathologic correlation. Radiographics 24:1477–1481
Bhattacharyya AK, Perrin R, Guha A (2004) Peripheral nerve tumors: management strategies and molecular insights. J Neurooncol 69:335–349
Canvasser DA, Naunheim KS (1996) Thoracoscopic management of posterior mediastinal tumors. Chest Surg Clin N Am 6:53–67
Evans DG (2009) Neurofibromatosis type 2 (NF2): a clinical and molecular review. Orphanet J Rare Dis 4:16
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
Evans DG, Mason S, Huson SM, Ponder M, Harding AE, Strachan T (1997) Spinal and cutaneous schwannomatosis is a variant form of type 2 neurofibromatosis: a clinical and molecular study. J Neurol Neurosurg Psychiatry 62:361–366
Evans DG, Baser ME, O’Reilly B, Rowe J, Gleeson M, Saeed S, King A, Huson SM, Kerr R, Thomas N, Irving R, MacFarlane R, Ferner R, McLeod R, Moffat D, Ramsden R (2005) Management of the patient and family with neurofibromatosis 2: a consensus conference statement. Br J Neurosurg 19:5–12
Grillo HC, Ojemann RG, Scannell JG, Zervas NT (1983) Combined approach to “dumbbell” intrathoracic and intraspinal neurogenic tumors. Ann Thorac Surg 36:402–407
Hajioff D, Raut VV, Walsh RM, Bath AP, Bance ML, Guha A, Tator CH, Rutka JA (2008) Conservative management of vestibular schwannomas: third review of a 10-year prospective study. Clin Otolaryngol 33:255–259
Jacoby LB, Jones D, Davis K, Kronn D, Short MP, Gusella J, MacCollin M (1997) Molecular analysis of the NF2 tumor-suppressor gene in schwannomatosis. Am J Hum Genet 61:1293–1302
Kehoe NJ, Reid RP, Semple JC (1995) Solitary benign peripheral-nerve tumours. Review of 32 years’ experience. J Bone Joint Surg Br 77:497–500
MacCollin M, Willett C, Heinrich B, Jacoby LB, Acierno JS Jr, Perry A, Louis DN (2003) Familial schwannomatosis: exclusion of the NF2 locus as the germline event. Neurology 60:1968–1974
MacCollin M, Chiocca EA, Evans DG, Friedman JM, Horvitz R, Jaramillo D, Lev M, Mautner VF, Niimura M, Plotkin SR, Sang CN, Stemmer-Rachamimov A, Roach ES (2005) Diagnostic criteria for schwannomatosis. Neurology 64:1838–1845
Martin TP, Tzifa K, Kowalski C, Holder RL, Walsh R, Irving RM (2008) Conservative versus primary surgical treatment of acoustic neuromas: a comparison of rates of facial nerve and hearing preservation. Clin Otolaryngol 33:228–235
Mathieu D, Kondziolka D, Flickinger JC, Niranjan A, Williamson R, Martin JJ, Lunsford LD (2007) Stereotactic radiosurgery for vestibular schwannomas in patients with neurofibromatosis type 2: an analysis of tumor control, complications, and hearing preservation rates. Neurosurgery 60:460–470
Mautner VF, Tatagiba M, Guthoff R, Samii M, Pulst SM (1993) Neurofibromatosis 2 in the pediatric age group. Neurosurgery 33:92–96
McClatchey AI (2007) Neurofibromatosis. Annu Rev Pathol 2:191–216
Mejico LJ (2010) Nonvestibulocochlear cranial nerve schwannomas. Curr Treat Options Neurol 12:37–42
Rousseau G, Noguchi T, Bourdon V, Sobol H, Olschwang S (2011) SMARCB1/INI1 germline mutations contribute to 10 % of sporadic schwannomatosis. BMC Neurol 11:9
Rowe JG, Radatz MW, Walton L, Soanes T, Rodgers J, Kemeny AA (2003) Clinical experience with gamma knife stereotactic radiosurgery in the management of vestibular schwannomas secondary to type 2 neurofibromatosis. J Neurol Neurosurg Psychiatry 74:1288–1293
Sakai F, Sone S, Kiyono K, Maruyama A, Ueda H, Aoki J, Kawai T, Ishii K, Morimoto M, Haniuda M et al (1992) Intrathoracic neurogenic tumors: MR-pathologic correlation. AJR Am J Roentgenol 159:279–283
Seppala MT, Haltia MJ, Sankila RJ, Jaaskelainen JE, Heiskanen O (1995) Long-term outcome after removal of spinal schwannoma: a clinicopathological study of 187 cases. J Neurosurg 83:621–626
Showalter TN, Werner-Wasik M, Curran WJ Jr, Friedman DP, Xu X, Andrews DW (2008) Stereotactic radiosurgery and fractionated stereotactic radiotherapy for the treatment of nonacoustic cranial nerve schwannomas. Neurosurgery 63:734–740
Sobel RA (1993) Vestibular (acoustic) schwannomas: histologic features in neurofibromatosis 2 and in unilateral cases. J Neuropathol Exp Neurol 52:106–113
Strollo DC, Rosado-de-Christenson ML, Jett JR (1997) Primary mediastinal tumors: part II. Tumors of the middle and posterior mediastinum. Chest 112:1344–1357
Zierold D, Halow KD (2000) Thoracoscopic resection as the preferred approach to posterior mediastinal neurogenic tumors. Surg Laparosc Endosc Percutan Tech 10:222–225
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2014 Springer Science+Business Media Dordrecht
About this chapter
Cite this chapter
Eguchi, T. et al. (2014). Multiple Schwannomas: Diagnosis and Treatment. In: Hayat, M. (eds) Tumors of the Central Nervous System, Volume 11. Tumors of the Central Nervous System, vol 11. Springer, Dordrecht. https://doi.org/10.1007/978-94-007-7037-9_27
Download citation
DOI: https://doi.org/10.1007/978-94-007-7037-9_27
Published:
Publisher Name: Springer, Dordrecht
Print ISBN: 978-94-007-7036-2
Online ISBN: 978-94-007-7037-9
eBook Packages: MedicineMedicine (R0)