Skip to main content

Advertisement

SpringerLink
Log in

Schwannoma of the trochlear nerve—an illustrated case series and a systematic review of management

  • Review
  • Published:
Neurosurgical Review Aims and scope Submit manuscript

Abstract

Schwannomas of cranial nerves in the absence of systemic neurofibromatosis are relatively rare. Among these, schwannomas of the trochlear nerve are even less common. They can be found incidentally or when they cause diplopia or other significant neurological deficits. Treatment options include observation only, neuro-ophthalmological intervention, and/or neurosurgical management via resection or sterotactic radiosurgery (SRS). In recent years, the latter has become an attractive therapeutic tool for a number of benign skull base neoplasm including a small number of reports on its successful use for trochlear Schwannomas. However, no treatment algorithm for the management of these tumors has been proposed so far. The goal of this manuscript is to illustrate a case series of this rare entity and to suggest a rational treatment algorithm for trochlear schwannomas, based on our institutional experience of recent cases, and a pertinent review of the literature. Including our series of 5 cases, a total of 85 cases reporting on the management of trochlear schwannomas have been published. Of those reported, less than half (40 %) of patients underwent surgical resection, whereas the remainder were managed conservatively or with SRS. Seventy-six percent (65/85) of the entire cohort presented with diplopia, which was the solitary symptom in over half of the cases (n = 39). All patients who presented with symptoms other than diplopia or headaches as solitary symptoms underwent surgical resection. Patients in the non-surgical group were mostly male (M/F = 3.5:1), presented at an older age and had shorter mean diameter (4.6 vs. 30.4 mm, p < 0.0001) when compared to the surgical group. Twelve patients in the entire cohort were treated with SRS, none of whom had undergone surgical resection before or after radiation treatment. Trochlear schwannoma patients without systemic neurofibromatosis are rare and infrequently reported in the literature. Of those, patients harboring symptomatic trochlear Schwannomas do not form a single homogenous group, but fall into two rather distinct subgroups regarding demographics and clinical characteristics. Among those patients in need of intervention, open microsurgical resection as well as less invasive treatment options exist, which all aim at safe relief of symptoms and prevention of progression. Both open microsurgical removal as well as SRS can achieve good long-term local control. Consequently, a tailored multidisciplinary treatment algorithm, based on the individual presentation and tumor configuration, is proposed.

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
Fig. 2
Fig. 3
Fig. 4
Fig. 5
Fig. 6

Similar content being viewed by others

References

  1. Abe T, Iwata T, Shimazu M, Iida M, Izumiyama H, Matsumoto K, Mizutani T, Tanaka H, Aruga T (1994) Two cases of trochlear nerve neurinoma. No Shinkei Geka 22:371–375 Japanese

    PubMed  CAS  Google Scholar 

  2. Abe T, Iwata T, Shimazu M, Matsumoto K (1994) Trochlear nerve neurinoma associated with a giant thrombosed dissecting aneurysm of the contralateral vertebral artery. Surg Neurol 42:438–441

    Article  PubMed  CAS  Google Scholar 

  3. Andrews DW, Werner-Wasik M, Den RB, Paek SH, Downes-Phillips B, Willcox TO, Bednarz G, Maltenfort M, Evans JJ, Curran WJ (2009) Toward dose optimization for fractionated stereotactic radiotherapy for acoustic neuromas: comparison of two dose cohorts. Int J Radiat Oncol Biol Phys 74:419–426

    Article  PubMed  Google Scholar 

  4. Arai H, Chichibu M, Ikuta F, Oyake Y (1969) Autopsy case of multiple primary glioma, meningioma and neurinoma and their origin. No To Shinkei 21:1241–1249 Japanese

    PubMed  CAS  Google Scholar 

  5. Bartalena T, Leoni C, Trossello MP, Rinaldi MF, Cianfoni A, Caprara G, Sverzellati N, De Filippo M, Padovani R (2010) Hourglass cystic schwannoma of the trochlear nerve. Acta Biomed 81:147–150

    PubMed  Google Scholar 

  6. Beppu T, Yoshida Y, Wada T, Arai H, Suzuki M, Kuroda K, Ogawa A (1997) Trochlear and abducens nerve neurinomas accompanied by a cerebellopontine angle meningioma—case report. Neurol Med Chir (Tokyo) 37:416–421 Japanese

    Article  CAS  Google Scholar 

  7. Boggan JE, Rosenblum ML, Wilson CB (1979) Neurilemmoma of the fourth cranial nerve. J Neurosurg 50:519–521

    Article  PubMed  CAS  Google Scholar 

  8. Boucher AB, Michael LM (2014) The middle fossa approach for the removal of a trochlear schwannoma. Case Rep Neurol Med 2014:1–6.

  9. Celli P, Ferrante L, Acqui M, Mastronardi L, Fortuna A, Palma L (1992) Neurinoma of the third, fourth, and sixth cranial nerves: a survey and report of a new fourth nerve case. Surg Neurol 38:216–224

    Article  PubMed  CAS  Google Scholar 

  10. Dolenc V, Coscia S (1996) Cystic trochlear nerve neurinoma. Br J of Neurosurg 10:593–598

    Article  CAS  Google Scholar 

  11. Du R, Dhoot J, McDermott MW, Gupta N (2003) Cystic schwannoma of the anterior tentorial hiatus. Pediat Neurosurg 38:167–173

    Article  Google Scholar 

  12. Elflein HM, Thomke F, Muller-Forell W, Pitz S (2010) Trochlear palsies caused by isolated trochlear schwannomas. Strabismus 18:83–86

    Article  PubMed  CAS  Google Scholar 

  13. Elmalem VI, Younge BR, Biousse V, Leavitt JA, Moster ML, Warner J, Kupersmith MJ, Landau K, Brodsky MC, Frohman LP (2009) Clinical course and prognosis of trochlear nerve schwannomas. Ophthalmology 116:2011–2016

    Article  PubMed  Google Scholar 

  14. Feinberg AS, Newman NJ (1999) Schwannoma in patients with isolated unilateral trochlear nerve palsy. Am J Ophthalmol 127:183–188

    Article  PubMed  CAS  Google Scholar 

  15. Garen PD, Harper CG, Teo C, Johnston IH (1987) Cystic schwannoma of the trochlear nerve mimicking a brain-stem tumor. J Neurosurg 67:928–930

    Article  PubMed  CAS  Google Scholar 

  16. Gentry LR, Mehta RC, Appen RE, Weinstein JM (1991) MR imaging of primary trochlear nerve neoplasms. AJNR Am J Neuroradiol 12:707–713

    PubMed  CAS  Google Scholar 

  17. Gerganov V, Amir S, Koerbel A, Brandes A, Stan A, Madjid S (2007) Cystic trochlear nerve schwannoma. Case report. Surg Neurol 68:221–225

    Article  PubMed  Google Scholar 

  18. Hatae R, Miyazono M, Kohri R, Maeda K, Naito S (2014) Trochlear nerve schwannoma with intratumoral hemorrhage presenting with persistent hiccups: a case report. J Neurol Surg Rep 75:e183–e188

    Article  PubMed  PubMed Central  Google Scholar 

  19. Ho K-L (1981) Schwannoma of the trochlear nerve. J Neurosurg 55:132–135

    Article  PubMed  CAS  Google Scholar 

  20. Jackowski A, Weiner G, O'Reilly G (1994) Trochlear nerve schwannomas: a case report and literature review. Br J Neurosurg 8:219–223

    Article  PubMed  CAS  Google Scholar 

  21. Kim IY, Kondziolka D, Niranjan A, Flickinger JC, Lunsford LD (2008) Gamma Knife surgery for schwannomas originating from cranial nerves III, IV, and VI. J Neurosurg 109(Suppl):149–153

    PubMed  Google Scholar 

  22. King JS (1976) Trochlear nerve sheath tumor. J Neurosurg 44:245–247

    Article  PubMed  CAS  Google Scholar 

  23. Kohama M, Murakami K, Endo T, Watanabe M, Tominaga T (2009) Surgical and histological observations of trochlear neurinoma. Neurol Med Chir 49:217–220 Japanese

    Article  Google Scholar 

  24. Kondziolka D, Lunsford LD, McLaughlin MR, Flickinger JC (1998) Long-term outcomes after radiosurgery for acoustic neuromas. N Engl J Med 339:1426–1433

    Article  PubMed  CAS  Google Scholar 

  25. Kondziolka D, Mathieu D, Lunsford LD, Martin JJ, Madhok R, Niranjan A, Flickinger JC (2008) Radiosurgery as definitive management of intracranial meningiomas. Neurosurgery 62:53–60

    Article  PubMed  Google Scholar 

  26. Kurita H, Sasaki T, Kawamoto S, Taniguchi M, Terahara A, Tago M, Kirino T (2009) Role of radiosurgery in the management of cavernous sinus meningiomas. Acta Neurol Scand 96:297–304

    Article  Google Scholar 

  27. Leunda G, Vaquero J, Cabezudo J, Garcia-Uria J, Bravo G (1982) Schwannoma of the oculomotor nerves. J Neurosurg 57:563–565

    Article  PubMed  CAS  Google Scholar 

  28. Lunsford LD, Niranjan A, Flickinger JC, Maitz A, Kondziolka D (2005) Radiosurgery of vestibular schwannomas: summary of experience in 829 cases. J Neurosurg 102:195–199

    Article  PubMed  Google Scholar 

  29. Mahadevan A, Floyd S, Wong E, Chen C, Kasper E (2011) Clinical outcome after Hypofractionated stereotactic radiotherapy (HSRT) for benign skull base tumors. Comput Aided Surg 16:112–120

    Article  PubMed  Google Scholar 

  30. Matsui T, Morikawa E, Morimoto T, Asano T (2002) Presigmoid transpetrosal approach for the treatment of a large trochlear nerve schwannoma. Case report. Neurol Med Chir 42:31–35 Japanese

    Article  Google Scholar 

  31. Maurice-Williams RS (1989) Isolated schwannoma of the fourth cranial nerve: case report. J Neurol Neurosurg Psychiatry 52:1442–1443

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  32. Mautner V-F, Lindenau M, Baser ME, Hazim W, Tatagiba M, Haase W, Samii M, Wais R, Pulst S-M (1996) The neuroimaging and clinical spectrum of neurofibromatosis 2. Neurosurgery 38:880–886

    Article  PubMed  CAS  Google Scholar 

  33. Minniti G, Amichetti M, Enrici R (2009) Radiotherapy and radiosurgery for benign skull base meningiomas. Radiat Oncol 4:42

    Article  PubMed  PubMed Central  Google Scholar 

  34. Murakawa T, SH UT, Yokoyama K, Takada M, Yamada H (1988) Trochlear neurinoma: case report. Tak Red Cross Hosp Kiyou 12:163–167 Japanese

    Google Scholar 

  35. Nadkarni TD, Goel A (1999) Trochlear nerve neurinoma presenting as pathological laughter. Br J of Neurosurg 13:212–213

    Article  CAS  Google Scholar 

  36. Ohba S, Miwa T, Kawase T (2006) Trochlear nerve schwannoma with intratumoral hemorrhage: case report. Neurosurgery 58:E791.

  37. Petrela E, Hodge CJ, Hahn SS, Chung CT, Mejico LJ (2009) Stereotactic radiosurgery in two cases of presumed fourth cranial nerve schwannoma. J Neuroophthalmol 29:54–57

    Article  PubMed  Google Scholar 

  38. Samii M, Draf W, Lang J (1989) Surgery of the skull base, 1st edn. Springer Science + Business Media, Heidelberg

    Book  Google Scholar 

  39. Santoreneos S, Hanieh A, Jorgensen RE (1997) Trochlear nerve schwannomas occurring in patients without Neurofibromatosis: case report and review of the literature. Neurosurgery 41:282–287

    Article  PubMed  CAS  Google Scholar 

  40. Shenouda EF, Ghosh A, Coakham HB (2002) Trochlear nerve schwannoma removed by combined petrosal approach. Br J of Neurosurg 16:600–604

    Article  CAS  Google Scholar 

  41. Shenoy SN, Raja A (2004) Cystic trochlear nerve neurinoma mimicking intrinsic brainstem tumour. Br J of Neurosurg 18:183–186

    Article  CAS  Google Scholar 

  42. Shibamoto Y, Oda Y, Yamashita J, Takahashi M, Kikuchi H, Abe M (1994) The role of cerebrospinal fluid cytology in radiotherapy planning for intracranial germinoma. Int J Radiat Oncol Biol Phys 29:1089–1094

    Article  PubMed  CAS  Google Scholar 

  43. Tokuriki Y, Yamashita J, Kikuchi H, Asato R, Handa H (1988) Trochlear nerve neurinoma. Neurol Med Chir 28:70–73 Japanese

    Article  CAS  Google Scholar 

  44. Türe U, Ozduman K, Elmaci I, Necmettin Pamir M (2002) Infratentorial lateral supracerebellar approach for trochlear nerve schwannoma. J Clin Neurosci 9:595–598

    Article  PubMed  Google Scholar 

  45. Veshchev I, Spektor S (2002) Trochlear nerve neuroma manifested with intractable atypical facial pain: case report. Neurosurgery 50:889–892

    Article  PubMed  Google Scholar 

  46. Yamamoto M, Jimbo M, Ide M, Kubo O (1987) Trochlear neurinoma. Surg Neurol 28:287–290

    Article  PubMed  CAS  Google Scholar 

  47. Younes WM, Hermann EJ, Krauss JK (2012) Cisternal trochlear nerve schwannoma: improvement of diplopia after subtotal tumour excision. Br J of Neurosurg 26:107–109

    Article  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Surgery, Division of Ophthalmology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA, USA

    Nurhan Torun

  2. Department of Surgery, Division of Neurosurgery, Beth Israel Deaconess Medical Center, Harvard Medical School, West Campus, Lowry Medical Office Building, Suite 3B, 110 Francis St., Boston, MA, 02215, USA

    Yosef Laviv, Kianush Karimian Jazi & Ekkehard M. Kasper

  3. Department of Radiation Oncology, Department of Radiology/Division of Neuroradiology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA, USA

    Anand Mahadevan & Ekkehard M. Kasper

  4. Department of Radiology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA, USA

    Rafeeque A. Bhadelia

  5. Department of Pathology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA, USA

    Anderson Matthew

  6. Department of Ophthalmology and Pediatrics, New England Eye Center, Tufts Medical Center, Boston, MA, USA

    Mitchell Strominger

Authors
  1. Nurhan Torun
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Yosef Laviv
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Kianush Karimian Jazi
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Anand Mahadevan
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Rafeeque A. Bhadelia
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Anderson Matthew
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Mitchell Strominger
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Ekkehard M. Kasper
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Ekkehard M. Kasper.

Ethics declarations

Conflict of interest

The authors declare that they have no conflict of interest.

Funding

This work was not funded or financially supported.

Informed consent

Informed consent was obtained from all individual participants included in the study.

Ethical approval

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

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Torun, N., Laviv, Y., Jazi, K.K. et al. Schwannoma of the trochlear nerve—an illustrated case series and a systematic review of management. Neurosurg Rev 41, 699–711 (2018). https://doi.org/10.1007/s10143-016-0783-y

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10143-016-0783-y

Keywords

Search

Navigation