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.
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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
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
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
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
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
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
Boggan JE, Rosenblum ML, Wilson CB (1979) Neurilemmoma of the fourth cranial nerve. J Neurosurg 50:519–521
Boucher AB, Michael LM (2014) The middle fossa approach for the removal of a trochlear schwannoma. Case Rep Neurol Med 2014:1–6.
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
Dolenc V, Coscia S (1996) Cystic trochlear nerve neurinoma. Br J of Neurosurg 10:593–598
Du R, Dhoot J, McDermott MW, Gupta N (2003) Cystic schwannoma of the anterior tentorial hiatus. Pediat Neurosurg 38:167–173
Elflein HM, Thomke F, Muller-Forell W, Pitz S (2010) Trochlear palsies caused by isolated trochlear schwannomas. Strabismus 18:83–86
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
Feinberg AS, Newman NJ (1999) Schwannoma in patients with isolated unilateral trochlear nerve palsy. Am J Ophthalmol 127:183–188
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
Gentry LR, Mehta RC, Appen RE, Weinstein JM (1991) MR imaging of primary trochlear nerve neoplasms. AJNR Am J Neuroradiol 12:707–713
Gerganov V, Amir S, Koerbel A, Brandes A, Stan A, Madjid S (2007) Cystic trochlear nerve schwannoma. Case report. Surg Neurol 68:221–225
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
Ho K-L (1981) Schwannoma of the trochlear nerve. J Neurosurg 55:132–135
Jackowski A, Weiner G, O'Reilly G (1994) Trochlear nerve schwannomas: a case report and literature review. Br J Neurosurg 8:219–223
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
King JS (1976) Trochlear nerve sheath tumor. J Neurosurg 44:245–247
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
Kondziolka D, Lunsford LD, McLaughlin MR, Flickinger JC (1998) Long-term outcomes after radiosurgery for acoustic neuromas. N Engl J Med 339:1426–1433
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
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
Leunda G, Vaquero J, Cabezudo J, Garcia-Uria J, Bravo G (1982) Schwannoma of the oculomotor nerves. J Neurosurg 57:563–565
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
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
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
Maurice-Williams RS (1989) Isolated schwannoma of the fourth cranial nerve: case report. J Neurol Neurosurg Psychiatry 52:1442–1443
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
Minniti G, Amichetti M, Enrici R (2009) Radiotherapy and radiosurgery for benign skull base meningiomas. Radiat Oncol 4:42
Murakawa T, SH UT, Yokoyama K, Takada M, Yamada H (1988) Trochlear neurinoma: case report. Tak Red Cross Hosp Kiyou 12:163–167 Japanese
Nadkarni TD, Goel A (1999) Trochlear nerve neurinoma presenting as pathological laughter. Br J of Neurosurg 13:212–213
Ohba S, Miwa T, Kawase T (2006) Trochlear nerve schwannoma with intratumoral hemorrhage: case report. Neurosurgery 58:E791.
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
Samii M, Draf W, Lang J (1989) Surgery of the skull base, 1st edn. Springer Science + Business Media, Heidelberg
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
Shenouda EF, Ghosh A, Coakham HB (2002) Trochlear nerve schwannoma removed by combined petrosal approach. Br J of Neurosurg 16:600–604
Shenoy SN, Raja A (2004) Cystic trochlear nerve neurinoma mimicking intrinsic brainstem tumour. Br J of Neurosurg 18:183–186
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
Tokuriki Y, Yamashita J, Kikuchi H, Asato R, Handa H (1988) Trochlear nerve neurinoma. Neurol Med Chir 28:70–73 Japanese
Türe U, Ozduman K, Elmaci I, Necmettin Pamir M (2002) Infratentorial lateral supracerebellar approach for trochlear nerve schwannoma. J Clin Neurosci 9:595–598
Veshchev I, Spektor S (2002) Trochlear nerve neuroma manifested with intractable atypical facial pain: case report. Neurosurgery 50:889–892
Yamamoto M, Jimbo M, Ide M, Kubo O (1987) Trochlear neurinoma. Surg Neurol 28:287–290
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
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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
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DOI: https://doi.org/10.1007/s10143-016-0783-y