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Neurofibromatosis type 2 associated with multiple cranial nerve schwannomas: a case report

Year 2007, Volume: 17 Issue: 3, 171 - 175, 23.05.2007

Abstract

A 16-year-old male patient complained of right-sided tinnitus and mild deafness of one-month history. He also had a family history of neurofibromatosis type 2 and a history of a prior operation for left vestibular schwannoma a year ago. Otoneurologic examination revealed moderate sensorineural hearing loss.Magnetic resonance imaging demonstrated multiple extra-axial enhancing masses in the vicinity of both hypoglossal nerves, the right vestibular nerve, the left vestibular nerve, the right trigeminal, the left occulo- motor and the right abducens nerves. These findings were evaluated as multiple cranial nerve schwanno- mas. The case was considered a rare manifestation of neurofibromatosis type 2 without any concomitant abnormality in the central nervous system.Symptomatic medical treatment was initiated and the patient was referred to the neurosurgery department.

References

  • Egelhoff JC, Bates DJ, Ross JS, Rothner AD, Cohen BH. Spinal MR findings in neurofibromatosis types 1 and 2. AJNR Am J Neuroradiol 1992;13:1071-7.
  • Evans DG, Huson SM, Donnai D, Neary W, Blair V, Newton V, et al. A clinical study of type 2 neurofibro- matosis. Q J Med 1992;84:603-18.
  • Halliday AL, Sobel RA, Martuza RL. Benign spinal nerve sheath tumors: their occurrence sporadically and in neurofibromatosis types 1 and 2. J Neurosurg 1991;74:248-53.
  • Mautner VF, Tatagiba M, Lindenau M, Funsterer C, Pulst SM, Baser ME, et al. Spinal tumors in patients with neurofibromatosis type 2: MR imaging study of frequency, multiplicity, and variety. AJR Am J Roentgenol 1995;165:951-5.
  • Neurofibromatosis. Conference statement. National Institutes of Health Consensus Development Conference. Arch Neurol 1988;45:575-8.
  • Louis DN, Ramesh V, Gusella JF. Neuropathology and molecular genetics of neurofibromatosis 2 and related tumors. Brain Pathol 1995;5:163-72.
  • Rouleau GA, Wertelecki W, Haines JL, Hobbs WJ, Trofatter JA, Seizinger BR, et al. Genetic linkage of bilateral acoustic neurofibromatosis to a DNA marker on chromosome 22. Nature 1987;329:246-8.
  • Gutmann DH, Aylsworth A, Carey JC, Korf B, Marks J, Pyeritz RE, et al. The diagnostic evaluation and multi- disciplinary management of neurofibromatosis 1 and neurofibromatosis 2. JAMA 1997;278:51-7.
  • Parry DM, Eldridge R, Kaiser-Kupfer MI, Bouzas EA, Pikus A, Patronas N. Neurofibromatosis 2 (NF2): clin- ical characteristics of 63 affected individuals and clini- cal evidence for heterogeneity. Am J Med Genet 1994; 52:450-61.
  • Evans DG, Mason S, Huson SM, Ponder M, Harding AE, Strachan T. Spinal and cutaneous schwannomato- sis is a variant form of type 2 neurofibromatosis: a clin- ical and molecular study. J Neurol Neurosurg Psychiatry 1997;62:361-6.
  • Evans DG, Blair V, Strachan T, Lye RH, Ramsden RT. Variation of expression of the gene for type 2 neurofi- bromatosis: absence of a gender effect on vestibular schwannomas, but confirmation of a preponderance of meningiomas in females. J Laryngol Otol 1995;109:830-5.
  • Karamitopoulou E, Perentes E, Diamantis I, Maraziotis T. Ki-67 immunoreactivity in human central nervous system tumors: a study with MIB 1 monoclonal anti- body on archival material. Acta Neuropathol 1994; 87:47-54.
  • Russell DS, Rubinstein LJ. Tumours of the cranial, spinal and peripheral nerve sheaths. In: Russel DS, Rubinstein LJ, editors. Pathology of the tumours of the nervous system. 5th ed. London: Edward Arnold; 1989. p. 533-89.
  • Woodruff JM, Kourea HP, Louis DN. Schwannoma. In: Kleihues P, Cavenee WK, editors. Pathology and genetics of tumours of the nervous system. Lyon: International Agency for Research on Cancer; 1997. p. 126-8.
  • Seppala M. Long-term outcome of surgery for spinal nerve sheath neoplasms [Dissertation]. University of Helsinki; 1998.
  • Kuratsu J, Ushio Y. Epidemiological study of primary intracranial tumors: a regional survey in Kumamoto Prefecture in the southern part of Japan. J Neurosurg 1996;84:946-50.
  • Preston-Martin S. Descriptive epidemiology of prima- ry tumors of the brain, cranial nerves and cranial meninges in Los Angeles County. Neuroepidemiology 1989;8:283-95.
  • Radhakrishnan K, Mokri B, Parisi JE, O’Fallon WM, Sunku J, Kurland LT. The trends in incidence of pri- mary brain tumors in the population of Rochester, Minnesota. Ann Neurol 1995;37:67-73.
  • Sobel RA. Vestibular (acoustic) schwannomas: histo- logic features in neurofibromatosis 2 and in unilateral cases. J Neuropathol Exp Neurol 1993;52:106-13.
  • Seppala MT, Sainio MA, Haltia MJ, Kinnunen JJ, Setala KH, Jaaskelainen JE. Multiple schwannomas: schwan- nomatosis or neurofibromatosis type 2? J Neurosurg 1998;89:36-41.
  • Minamino M, Iwai H, Yano J, Fujisawa T, Yamashita T. Neurofibromatosis type 2 associated with cranial nerve schwannomas. Otolaryngol Head Neck Surg 2001;124:581-3.
  • Suresh TN, Mahadevan A, Chandrashekhar Sagar B, Santosh V, Yasha TC, Shankar SK. Unusual case of mul- tiple cellular and malignant schwannomas of the cranial and spinal nerves. Clin Neuropathol 2003;22:23-9.
  • Kuchna I, Zabek M, Dambska M, Matyja E, Wierzba- Bobrowicz T. Neurofibromatosis type 2. Case report. Folia Neuropathol 1995;33:141-4.
  • Black FO, Brackmann DE, Hitselberger WE, Purdy J. Preservation of auditory and vestibular function after surgical removal of bilateral vestibular schwannomas in a patient with neurofibromatosis type 2. Am J Otol 1995;16:431-43.
  • Blomstedt GC, Jaaskelainen JE, Pyykko I, Ishizaki H, Troupp H, Palva T. Recovery of the sutured facial nerve after removal of acoustic neuroma in patients with neu- rofibromatosis-2. Neurosurgery 1994;35:364-8.
  • Samii M, Matthies C, Tatagiba M. Management of vestibular schwannomas (acoustic neuromas): audito- ry and facial nerve function after resection of 120 vestibular schwannomas in patients with neurofibro- matosis 2. Neurosurgery 1997;40:696-705.
  • Wigand ME, Haid T, Goertzen W, Wolf S. Preservation of hearing in bilateral acoustic neurinomas by deliber- ate partial resection. Acta Otolaryngol 1992;112:237-41.

Kraniyal sinir flivannomlar›n›n efllik etti¤i tip 2 nörofibromatozis: Olgu sunumu

Year 2007, Volume: 17 Issue: 3, 171 - 175, 23.05.2007

Abstract

On altı yaşında erkek hasta, bir ay önce başlayan sağ taraflı kulak çınlaması ve hafif sağırlık yakın- malarıyla başvurdu. Aile öyküsünde nörofibromato- zis tip 2 bulunan hasta bir yıl önce sol vestibüler şivannom nedeniyle ameliyet geçirmişti. Otonörolo- jik muayenede orta derecede sensorinöral işitme kaybı saptandı. Manyetik rezonans görüntülemede, iki taraflı hipoglossal sinirler, sağ ve sol vestibüler sinirler, sağ trigeminal, sol okulomotor ve sağ ab- dusens sinirlerin komşuluğunda ekstra-aksiyel yer- leşim gösteren, kontrast tutan birçok kitle saptandı. Kitleler kraniyal sinir şivannomu olarak değerlendi- rildi. Bulgular, eşlik eden herhangi bir santral sinir sistemi anormalliğinin bulunmadığı, nörofibromato- zis tip 2’nin nadir görülen bir tablosu olarak kabul edildi. Semptomatik medikal tedaviye başlanan hasta beyin cerrahisi kliniğine sevk edildi

References

  • Egelhoff JC, Bates DJ, Ross JS, Rothner AD, Cohen BH. Spinal MR findings in neurofibromatosis types 1 and 2. AJNR Am J Neuroradiol 1992;13:1071-7.
  • Evans DG, Huson SM, Donnai D, Neary W, Blair V, Newton V, et al. A clinical study of type 2 neurofibro- matosis. Q J Med 1992;84:603-18.
  • Halliday AL, Sobel RA, Martuza RL. Benign spinal nerve sheath tumors: their occurrence sporadically and in neurofibromatosis types 1 and 2. J Neurosurg 1991;74:248-53.
  • Mautner VF, Tatagiba M, Lindenau M, Funsterer C, Pulst SM, Baser ME, et al. Spinal tumors in patients with neurofibromatosis type 2: MR imaging study of frequency, multiplicity, and variety. AJR Am J Roentgenol 1995;165:951-5.
  • Neurofibromatosis. Conference statement. National Institutes of Health Consensus Development Conference. Arch Neurol 1988;45:575-8.
  • Louis DN, Ramesh V, Gusella JF. Neuropathology and molecular genetics of neurofibromatosis 2 and related tumors. Brain Pathol 1995;5:163-72.
  • Rouleau GA, Wertelecki W, Haines JL, Hobbs WJ, Trofatter JA, Seizinger BR, et al. Genetic linkage of bilateral acoustic neurofibromatosis to a DNA marker on chromosome 22. Nature 1987;329:246-8.
  • Gutmann DH, Aylsworth A, Carey JC, Korf B, Marks J, Pyeritz RE, et al. The diagnostic evaluation and multi- disciplinary management of neurofibromatosis 1 and neurofibromatosis 2. JAMA 1997;278:51-7.
  • Parry DM, Eldridge R, Kaiser-Kupfer MI, Bouzas EA, Pikus A, Patronas N. Neurofibromatosis 2 (NF2): clin- ical characteristics of 63 affected individuals and clini- cal evidence for heterogeneity. Am J Med Genet 1994; 52:450-61.
  • Evans DG, Mason S, Huson SM, Ponder M, Harding AE, Strachan T. Spinal and cutaneous schwannomato- sis is a variant form of type 2 neurofibromatosis: a clin- ical and molecular study. J Neurol Neurosurg Psychiatry 1997;62:361-6.
  • Evans DG, Blair V, Strachan T, Lye RH, Ramsden RT. Variation of expression of the gene for type 2 neurofi- bromatosis: absence of a gender effect on vestibular schwannomas, but confirmation of a preponderance of meningiomas in females. J Laryngol Otol 1995;109:830-5.
  • Karamitopoulou E, Perentes E, Diamantis I, Maraziotis T. Ki-67 immunoreactivity in human central nervous system tumors: a study with MIB 1 monoclonal anti- body on archival material. Acta Neuropathol 1994; 87:47-54.
  • Russell DS, Rubinstein LJ. Tumours of the cranial, spinal and peripheral nerve sheaths. In: Russel DS, Rubinstein LJ, editors. Pathology of the tumours of the nervous system. 5th ed. London: Edward Arnold; 1989. p. 533-89.
  • Woodruff JM, Kourea HP, Louis DN. Schwannoma. In: Kleihues P, Cavenee WK, editors. Pathology and genetics of tumours of the nervous system. Lyon: International Agency for Research on Cancer; 1997. p. 126-8.
  • Seppala M. Long-term outcome of surgery for spinal nerve sheath neoplasms [Dissertation]. University of Helsinki; 1998.
  • Kuratsu J, Ushio Y. Epidemiological study of primary intracranial tumors: a regional survey in Kumamoto Prefecture in the southern part of Japan. J Neurosurg 1996;84:946-50.
  • Preston-Martin S. Descriptive epidemiology of prima- ry tumors of the brain, cranial nerves and cranial meninges in Los Angeles County. Neuroepidemiology 1989;8:283-95.
  • Radhakrishnan K, Mokri B, Parisi JE, O’Fallon WM, Sunku J, Kurland LT. The trends in incidence of pri- mary brain tumors in the population of Rochester, Minnesota. Ann Neurol 1995;37:67-73.
  • Sobel RA. Vestibular (acoustic) schwannomas: histo- logic features in neurofibromatosis 2 and in unilateral cases. J Neuropathol Exp Neurol 1993;52:106-13.
  • Seppala MT, Sainio MA, Haltia MJ, Kinnunen JJ, Setala KH, Jaaskelainen JE. Multiple schwannomas: schwan- nomatosis or neurofibromatosis type 2? J Neurosurg 1998;89:36-41.
  • Minamino M, Iwai H, Yano J, Fujisawa T, Yamashita T. Neurofibromatosis type 2 associated with cranial nerve schwannomas. Otolaryngol Head Neck Surg 2001;124:581-3.
  • Suresh TN, Mahadevan A, Chandrashekhar Sagar B, Santosh V, Yasha TC, Shankar SK. Unusual case of mul- tiple cellular and malignant schwannomas of the cranial and spinal nerves. Clin Neuropathol 2003;22:23-9.
  • Kuchna I, Zabek M, Dambska M, Matyja E, Wierzba- Bobrowicz T. Neurofibromatosis type 2. Case report. Folia Neuropathol 1995;33:141-4.
  • Black FO, Brackmann DE, Hitselberger WE, Purdy J. Preservation of auditory and vestibular function after surgical removal of bilateral vestibular schwannomas in a patient with neurofibromatosis type 2. Am J Otol 1995;16:431-43.
  • Blomstedt GC, Jaaskelainen JE, Pyykko I, Ishizaki H, Troupp H, Palva T. Recovery of the sutured facial nerve after removal of acoustic neuroma in patients with neu- rofibromatosis-2. Neurosurgery 1994;35:364-8.
  • Samii M, Matthies C, Tatagiba M. Management of vestibular schwannomas (acoustic neuromas): audito- ry and facial nerve function after resection of 120 vestibular schwannomas in patients with neurofibro- matosis 2. Neurosurgery 1997;40:696-705.
  • Wigand ME, Haid T, Goertzen W, Wolf S. Preservation of hearing in bilateral acoustic neurinomas by deliber- ate partial resection. Acta Otolaryngol 1992;112:237-41.
There are 27 citations in total.

Details

Primary Language English
Journal Section Case Report
Authors

Ahmet Mesrur Halefoğlu This is me

Publication Date May 23, 2007
Published in Issue Year 2007 Volume: 17 Issue: 3

Cite

APA Halefoğlu, A. M. (2007). Neurofibromatosis type 2 associated with multiple cranial nerve schwannomas: a case report. The Turkish Journal of Ear Nose and Throat, 17(3), 171-175.
AMA Halefoğlu AM. Neurofibromatosis type 2 associated with multiple cranial nerve schwannomas: a case report. Tr-ENT. May 2007;17(3):171-175.
Chicago Halefoğlu, Ahmet Mesrur. “Neurofibromatosis Type 2 Associated With Multiple Cranial Nerve Schwannomas: A Case Report”. The Turkish Journal of Ear Nose and Throat 17, no. 3 (May 2007): 171-75.
EndNote Halefoğlu AM (May 1, 2007) Neurofibromatosis type 2 associated with multiple cranial nerve schwannomas: a case report. The Turkish Journal of Ear Nose and Throat 17 3 171–175.
IEEE A. M. Halefoğlu, “Neurofibromatosis type 2 associated with multiple cranial nerve schwannomas: a case report”, Tr-ENT, vol. 17, no. 3, pp. 171–175, 2007.
ISNAD Halefoğlu, Ahmet Mesrur. “Neurofibromatosis Type 2 Associated With Multiple Cranial Nerve Schwannomas: A Case Report”. The Turkish Journal of Ear Nose and Throat 17/3 (May 2007), 171-175.
JAMA Halefoğlu AM. Neurofibromatosis type 2 associated with multiple cranial nerve schwannomas: a case report. Tr-ENT. 2007;17:171–175.
MLA Halefoğlu, Ahmet Mesrur. “Neurofibromatosis Type 2 Associated With Multiple Cranial Nerve Schwannomas: A Case Report”. The Turkish Journal of Ear Nose and Throat, vol. 17, no. 3, 2007, pp. 171-5.
Vancouver Halefoğlu AM. Neurofibromatosis type 2 associated with multiple cranial nerve schwannomas: a case report. Tr-ENT. 2007;17(3):171-5.