Skip to main content
SpringerLink
Log in

Management of non-vestibular schwannomas in adult patients: a systematic review and consensus statement on behalf of the EANS skull base section Part III: Lower cranial nerve schwannomas, jugular foramen (CN IX, X, XI) and hypoglossal schwannoma (XII)

  • Original Article - Tumor - Schwannoma
  • Published:
Acta Neurochirurgica Aims and scope Submit manuscript

Abstract

Background

Non-vestibular schwannomas are relatively rare, with trigeminal and jugular foramen schwannomas being the most common. This is a heterogenous group which requires detailed investigation and careful consideration to management strategy. The optimal management for these tumours remains unclear, and there are several controversies. The aim of this paper is to provide insight into the main principles defining management and surgical strategy, in order to formulate a series of recommendations.

Methods

A task force was created by the EANS skull base section committee along with its members and other renowned experts in the field to generate recommendations for the surgical management of these tumours on a European perspective. To achieve this, the task force performed an extensive systematic review in this field and had discussions within the group. This article is the third of a three-part series describing non-vestibular schwannomas (IX, X, XI, XII).

Results

A summary of literature evidence was proposed after discussion within the EANS skull base section. The constituted task force dealt with the practice patterns that exist with respect to preoperative radiological investigations, ophthalmological assessments, optimal surgical and radiotherapy strategies and follow-up management.

Conclusion

This article represents the consensually derived opinion of the task force with respect to the treatment of non-vestibular schwannomas. For each of these tumours, the management paradigm is shifting towards the compromise between function preservation and progression free survival.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1

Similar content being viewed by others

References

  1. Aihara K, Morita A (2005) Dumbbell-shaped hypoglossal schwannoma in an elderly woman: a clinical dilemma. Surg Neurol. https://doi.org/10.1016/j.surneu.2004.07.045

    Article  PubMed  Google Scholar 

  2. Bartal AD, Djaldetti MM, Mandel EM, Lerner MA (1973) Dumb bell neurinoma of the hypoglossal nerve. J Neurol Neurosurg Psychiatry. https://doi.org/10.1136/jnnp.36.4.592

    Article  PubMed  PubMed Central  Google Scholar 

  3. Berger MS, Edwards MSB, Bingham WG (1982) Hypoglossal neurilemoma: case report and review of the literature. Neurosurgery. https://doi.org/10.1227/00006123-198205000-00013

    Article  PubMed  Google Scholar 

  4. Bindal S, El Ahmadieh TY, Plitt A, Aoun SG, Neeley OJ, El Tecle NE, Barnett S, Gluf W (2019) Hypoglossal schwannomas: a systematic review of the literature. J Clin Neurosci. https://doi.org/10.1016/j.jocn.2018.11.037

    Article  PubMed  Google Scholar 

  5. Bulsara KR, Sameshima T, Friedman AH, Fukushima T (2008) Microsurgical management of 53 jugular foramen schwannomas: lessons learned incorporated into a modified grading system - Clinical article. J Neurosurg. https://doi.org/10.3171/JNS/2008/109/11/0794

    Article  PubMed  Google Scholar 

  6. Carvalho GA, Tatagiba M, Samii M (2000) Cystic schwannomas of the jugular foramen: clinical and surgical remarks. Neurosurgery. https://doi.org/10.1097/00006123-200003000-00007

    Article  PubMed  Google Scholar 

  7. Chang KC, Leu YS (2002) Hypoglossal schwannoma in the submandibular space. J Laryngol Otol. https://doi.org/10.1258/0022215021910177

    Article  PubMed  Google Scholar 

  8. Chibbaro S, Mirone G, Makiese O, Bresson D, George B (2009) Dumbbell-shaped jugular foramen schwannomas: surgical management, outcome and complications on a series of 16 patients. Neurosurg Rev. https://doi.org/10.1007/s10143-009-0188-2

    Article  PubMed  Google Scholar 

  9. D.D. C, N.L. M, K. V, S. S-A, R.W. P, N. T, V.K.H. S, C.A. D, R.F. S (2011) Surgical management and outcome of schwannomas in the craniocervical region: clinical article. J. Neurosurg.

  10. Dolan EJ, Tucker WS, Rotenberg D, Chui M (1982) Intracranial hypoglossal schwannoma as an unusual cause of facial nerve palsy Case report. J Neurosurg. https://doi.org/10.3171/jns.1982.56.3.0420

    Article  PubMed  Google Scholar 

  11. Eldevik OP, Gabrielsen TO, Jacobsen EA (2000) Imaging findings schwannomas of the jugular foramen. Am. J. Neuroradiol.

  12. Elsharkawy M, Xu Z, Schlesinger D, Sheehan JP (2012) Gamma Knife surgery for nonvestibular schwannomas: radiological and clinical outcomes: clinical article. J Neurosurg. https://doi.org/10.3171/2011.8.JNS11215

    Article  PubMed  Google Scholar 

  13. Goel A, Desai K (2000) Cystic Schwannomas of the jugular foramen: clinical and surgical remarks. Neurosurgery. https://doi.org/10.1093/neurosurgery/47.6.1470

    Article  PubMed  Google Scholar 

  14. Hasegawa T, Kato T, Kida Y et al (2016) Gamma Knife surgery for patients with jugular foramen schwannomas: a multiinstitutional retrospective study in Japan. J Neurosurg. https://doi.org/10.3171/2015.8.JNS151156

    Article  PubMed  Google Scholar 

  15. Ichimura S, Yoshida K, Kawase T (2009) Surgical approach for hypoglossal schwannomas to prevent deformity of the atlanto-occipital joint. Acta Neurochir (Wien). https://doi.org/10.1007/s00701-009-0284-7

    Article  Google Scholar 

  16. Ichimura S, Yoshida K, Sutiono AB, Horiguchi T, Sasaki H, Kawase T (2010) Greater petrosal nerve schwannomas—analysis of four cases and review of the literature. Neurosurg Rev. https://doi.org/10.1007/s10143-010-0277-2

    Article  PubMed  Google Scholar 

  17. Inoue T, Nonaka Y, Hirai H, Shima A, Suzuki F, Matsuda M, Fukushima T (2018) Single-stage total resection of giant dumbbell-shaped hypoglossal schwannoma: a case report. Acta Neurochir (Wien). https://doi.org/10.1007/s00701-017-3431-6

    Article  Google Scholar 

  18. Jean WC, Felbaum D (2016) Evolution of the intracranial approaches to jugular foramen tumors: a surgeon’s personal perspective through three illustrative cases. Cureus. https://doi.org/10.7759/cureus.530

    Article  PubMed  PubMed Central  Google Scholar 

  19. Kadri PAS, Al-Mefty O (2004) Surgical treatment of dumbbell-shaped jugular foramen schwannomas. Neurosurg Focus. https://doi.org/10.3171/foc.2004.17.2.9

    Article  PubMed  Google Scholar 

  20. Kankane VK, Warade AC, Misra BK (2019) Nonvestibular schwannoma tumors in the cerebellopontine angle: a single-surgeon experience. Asian J Neurosurg 14(1):154. https://doi.org/10.4103/ajns.ajns_335_17

    Article  PubMed  PubMed Central  Google Scholar 

  21. Kano H, Meola A, Yang HC et al (2018) Stereotactic radiosurgery for jugular foramen schwannomas: an international multicenter study. J Neurosurg. https://doi.org/10.3171/2017.5.JNS162894

    Article  PubMed  Google Scholar 

  22. Kaye AH, Hahn JF, Kinney SE, Hardy RW, Bay JW (1984) Jugular foramen schwannomas. J Neurosurg. https://doi.org/10.3171/jns.1984.60.5.1045

    Article  PubMed  Google Scholar 

  23. Kuo LT, Huang APH, Kuo KT, Tseng HM (2008) Extradural dumbbell schwannoma of the hypoglossal nerve: a case report with review of the literature. Surg Neurol. https://doi.org/10.1016/j.surneu.2007.11.023

    Article  PubMed  Google Scholar 

  24. Lee MK, Sidell DR, Mendelsohn AH, Blackwell KE (2012) Hypoglossal schwannoma masquerading as a carotid body tumor. Case Rep Otolaryngol. https://doi.org/10.1155/2012/842761

    Article  PubMed  PubMed Central  Google Scholar 

  25. Lee M, Tong K (2016) Jugular foramen schwannoma mimicking paraganglioma: case report and review of imaging findings. Radiol Case Reports. https://doi.org/10.1016/j.radcr.2015.12.002

    Article  Google Scholar 

  26. Li Y, Lou J, Qiu S, Guo Y, Pan M (2016) Hypofractionated stereotactic radiotherapy for dumbbell-shaped hypoglossal schwannomas: two cases of long-term follow-up and a review of the literature. Mol Clin Oncol. https://doi.org/10.3892/mco.2016.922

    Article  PubMed  PubMed Central  Google Scholar 

  27. Lunsford LD, Sheehan JP (2015) Intracranial stereotactic radiosurgery, 2nd edn. Thieme

    Google Scholar 

  28. Mathiesen T, Svensson M, Lundgren J, Kihlström L, Parisotto R, Bagger-Sjöbäck D (2009) Hypoglossal schwannoma—successful reinnervation and functional recovery of the tongue following tumour removal and nerve grafting. Acta Neurochir (Wien). https://doi.org/10.1007/s00701-009-0226-4

    Article  Google Scholar 

  29. Mazzoni A, Saleh E, Sanna M, Achilli V (1997) Lower cranial nerve schwannomas involving the jugular foramen. Ann Otol Rhinol Laryngol. https://doi.org/10.1177/000348949710600503

    Article  PubMed  Google Scholar 

  30. Nonaka Y, Grossi PM, Bulsara KR, Taniguchi RM, Friedman AH, Fukushima T (2011) Microsurgical management of hypoglossal schwannomas over 3 decades: a modified grading scale to guide surgical approach. Neurosurgery. https://doi.org/10.1227/NEU.0b013e31822a547b

    Article  PubMed  Google Scholar 

  31. Di Pascuale I, Brito N, Zerpa J, Roa C, Faria G (2020) Hypoglossal nerve schwannoma: case report and literature review. World Neurosurg. https://doi.org/10.1016/j.wneu.2019.12.006

    Article  PubMed  Google Scholar 

  32. Pellet W, Cannoni M, Pech A (1988) The widened transcochlear approach to jugular foramen tumors. J Neurosurg. https://doi.org/10.3171/jns.1988.69.6.0887

    Article  PubMed  Google Scholar 

  33. Ryu SM, Il LJ, Park K, Choi JW, Kong DS, Nam DH, Jeong HS, Cho YS, Seol HJ (2017) Optimal treatment of jugular foramen schwannomas: long-term outcome of a multidisciplinary approach for a series of 29 cases in a single institute. Acta Neurochir (Wien). https://doi.org/10.1007/s00701-017-3230-0

    Article  Google Scholar 

  34. Samii M, Alimohamadi M, Gerganov V (2015) Surgical treatment of jugular foramen schwannoma: surgical treatment based on a new classification. Neurosurgery. https://doi.org/10.1227/NEU.0000000000000831

    Article  PubMed  Google Scholar 

  35. Samii M, Babu RP, Tatagiba M, Sepehrnia A (1995) Surgical treatment of jugular foramen schwannomas. J Neurosurg. https://doi.org/10.3171/jns.1995.82.6.0924

    Article  PubMed  Google Scholar 

  36. Samii M, Migliori MM, Tatagiba M, Babu R (1995) Surgical treatment of trigeminal schwannomas. J Neurosurg. https://doi.org/10.3171/jns.1995.82.5.0711

    Article  PubMed  Google Scholar 

  37. Sanna M, Bacciu A, Falcioni M, Taibah A (2006) Surgical management of jugular foramen schwannomas with hearing and facial nerve function preservation: a series of 23 cases and review of the literature. Laryngoscope. https://doi.org/10.1097/01.mlg.0000246193.84319.e5

    Article  PubMed  Google Scholar 

  38. Santander XA, Cotúa CE, Saldaña C (2017) Spontaneous regression of a hypoglossal neurinoma: case report and review of the literature. World Neurosurg. https://doi.org/10.1016/j.wneu.2017.06.004

    Article  PubMed  Google Scholar 

  39. Sarma S, Sekhar LN, Schessel DA (2002) Nonvestibular schwannomas of the brain: a 7-year experience. Neurosurgery 50(3):437–449

    PubMed  Google Scholar 

  40. Sedney CL, Nonaka Y, Bulsara KR, Fukushima T (2013) Microsurgical management of jugular foramen schwannomas. Neurosurgery. https://doi.org/10.1227/NEU.0b013e3182770e74

    Article  PubMed  Google Scholar 

  41. Shinya Y, Hasegawa H, Shin M, Sugiyama T, Kawashima M, Katano A, Kashio A, Kondo K, Saito N (2021) Long-term outcomes of stereotactic radiosurgery for trigeminal facial and jugular foramen schwannoma in comparison with vestibular schwannoma. Cancers (Basel) 13(5):1140. https://doi.org/10.3390/cancers13051140

    Article  Google Scholar 

  42. Suri A, Bansal S, Sharma BS, Mahapatra AK, Kale SS, Chandra PS, Singh M, Kumar R, Sharma MS (2014) Management of hypoglossal schwannomas: single institutional experience of 14 cases. J Neurol Surgery, Part B Skull Base. https://doi.org/10.1055/s-0033-1356924

    Article  Google Scholar 

  43. Suri A, Bansal S, Singh M, Mahapatra AK, Sharma BS (2014) Jugular foramen schwannomas: a single institution patient series. J Clin Neurosci. https://doi.org/10.1016/j.jocn.2013.02.029

    Article  PubMed  Google Scholar 

  44. Sutay S, Tekinsoy B, Ceryan K, Aksu Y (1993) Submaxillary hypoglossal neurilemmoma. J Laryngol Otol. https://doi.org/10.1017/S0022215100124892

    Article  PubMed  Google Scholar 

  45. Sutiono AB, Kawase T, Tabuse M, Kitamura Y, Arifin MZ, Horiguchi T, Yoshida K (2011) Importance of preserved periosteum around jugular foramen neurinomas for functional outcome of lower cranial nerves: anatomic and clinical studies. Neurosurgery. https://doi.org/10.1227/NEU.0b013e31822a19a3

    Article  PubMed  Google Scholar 

  46. Tan LC, Bordi L, Symon L, Cheesman AD (1990) Jugular foramen neuromas: a review of 14 cases. Surg Neurol. https://doi.org/10.1016/0090-3019(90)90130-H

    Article  PubMed  Google Scholar 

  47. Wang X, Long W, Liu D, Yuan J, Xiao Q, Liu Q (2020) Optimal surgical approaches and treatment outcomes in patients with jugular foramen schwannomas: a single institution series of 31 cases and a literature review. Neurosurg Rev 43(5):1339–1350

    Article  Google Scholar 

  48. Weindling SM, Wood CP, Hoxworth JM (2017) Hypoglossal canal lesions: distinctive imaging features and simple diagnostic algorithm. Am J Roentgenol. https://doi.org/10.2214/AJR.17.18102

    Article  Google Scholar 

  49. Yasumatsu R, Nakashima T, Miyazaki R, Segawa Y, Komune S (2013) Diagnosis and management of extracranial head and neck schwannomas: a review of 27 cases. Int J Otolaryngol. https://doi.org/10.1155/2013/973045

    Article  PubMed  PubMed Central  Google Scholar 

  50. Zeng XJ, Li D, Hao SY et al (2016) Long-term functional and recurrence outcomes of surgically treated jugular foramen schwannomas: a 20-year experience. World Neurosurg. https://doi.org/10.1016/j.wneu.2015.09.104

    Article  PubMed  Google Scholar 

  51. Zhang Q, Kong F, Guo H, Chen G, Liang J, Li M, Ling F (2012) Surgical treatment of dumbbell-shaped hypoglossal schwannoma via a pure endoscopic transoral approach. Acta Neurochir (Wien). https://doi.org/10.1007/s00701-011-1193-0

    Article  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Neurosurgery, Barts Health NHS Trust, St. Bartholomew’s and The Royal London Hospital, London, UK

    Jarnail Bal & Dimitrios Paraskevopoulos

  2. Department of Neurosurgery, Vrije Universiteit Brussel (VUB), Universitair Ziekenhuis Brussel (UZ Brussel), Laarbeeklaan 101, 1090, Brussels, Belgium

    Michael Bruneau

  3. Neuro-Oncologic and Vascular Department, Hôpital Neurologique Pierre Wertheimer, Lyon, France

    Moncef Berhouma

  4. Department of Neurosurgery, Medical Faculty, Heinrich-Heine-University Düsseldorf, Düsseldorf, Germany

    Jan F. Cornelius

  5. Department of Neurosurgery, University Hospital of Naples Federico II, Napoli, Italy

    Luigi M. Cavallo

  6. Department of Neurosurgery, Lausanne University Hospital and University of Lausanne, 42 rue du Bugnon, 1011, Lausanne, Switzerland

    Roy T. Daniel & Mahmoud Messerer

  7. Department of Neurosurgery, Lariboisière Hospital, Paris, France

    Sebastien Froelich

  8. Department of Neurosurgery, Hôpital Neurologique Pierre Wertheimer, Lyon, France

    Emmanuel Jouanneau

  9. Department of Neurosurgery, University Hospital of Geneva, Geneva, Switzerland

    Torstein R. Meling

  10. Neurosurgery Department, APHM, Hopital Nord, Marseille, France

    Pierre-Hugues Roche

  11. Department of Neurosurgery, University Medicine Greifswald, Greifswald, Germany

    Henry Schroeder

  12. Department of Neurosurgery, Eberhard Karls University of Tübingen, Tübingen, Germany

    Marcos Tatagiba

  13. Department of Neurosurgery, Complejo Hospitalario de Navarra, Pamplona, Spain

    Idoya Zazpe

Authors
  1. Jarnail Bal
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Michael Bruneau
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Moncef Berhouma
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Jan F. Cornelius
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Luigi M. Cavallo
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Roy T. Daniel
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Sebastien Froelich
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Emmanuel Jouanneau
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. Torstein R. Meling
    View author publications

    You can also search for this author in PubMed Google Scholar

  10. Mahmoud Messerer
    View author publications

    You can also search for this author in PubMed Google Scholar

  11. Pierre-Hugues Roche
    View author publications

    You can also search for this author in PubMed Google Scholar

  12. Henry Schroeder
    View author publications

    You can also search for this author in PubMed Google Scholar

  13. Marcos Tatagiba
    View author publications

    You can also search for this author in PubMed Google Scholar

  14. Idoya Zazpe
    View author publications

    You can also search for this author in PubMed Google Scholar

  15. Dimitrios Paraskevopoulos
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Dimitrios Paraskevopoulos.

Ethics declarations

Ethics approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the local Ethical Committee (Geneva Ethics Committee Board no. 11-233R, NAC 11-085R) and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.

Consent to participate

For this retrospective type of study, formal consent is not required.

Conflict of interest

The authors declare no competing interests.

Additional information

Publisher's note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Comments

This is yet another excellent attempt by the EANS skull base section to produce a consensus statement and review the literature regarding the non-vestibular schwannomas in adults. I congratulate the Editor as well the EANS skull base section for this exercise. The statements made are reasonable, and this manuscript will be value addition to the literature. A more comprehensive review of the literature is desirable. We have published our operative experience with the management of 224 consecutive non-vestibular schwannoma C P Angle tumours between 2001 and 2014.(1) The series had 34 trigeminal schwannoma and 26 jugular foramen schwannomas. Treatment strategy of jugular foramen schwannoma is best individualised and depends on the age of the patient, size of the tumour and lower cranial nerve involvement. Retrosigmoid approach was employed in 77 per cent of cases, and gross total excision was achieved in 62 per cent of cases. We recommend a more radical approach with an attempt at total excision in young patients with large tumours, especially with compromised lower cranial nerve function and a more conservative approach leaving the tumour in the jugular foramen for adjunct radiosurgery in patients with intact lower cranial function preoperatively. A similar conservative approach is preferred in elderly patients and patients with co morbidity. A period of observation is recommended in elderly patients with small tumours or incidental pick up of tumours.

Basant Misra.

India.

Reference:

1. Kankane VK, Warade AC, Misra BK. Non-vestibular schwannoma tumors in the cerebellopontine angle: A single-surgeon experience. Asian J Neurosurg (2019) 10.4103/ajns.AJNS_335_17.

This article is part of the Topical Collection on Tumor - Schwannoma

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Bal, J., Bruneau, M., Berhouma, M. et al. Management of non-vestibular schwannomas in adult patients: a systematic review and consensus statement on behalf of the EANS skull base section Part III: Lower cranial nerve schwannomas, jugular foramen (CN IX, X, XI) and hypoglossal schwannoma (XII). Acta Neurochir 164, 321–329 (2022). https://doi.org/10.1007/s00701-021-05072-y

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00701-021-05072-y

Keyword

Search

Navigation