|LETTER TO EDITOR
|Year : 2016 | Volume
| Issue : 2 | Page : 167-168
Multiple bilateral cranial nerve schwannomas in a patient with neurofibromatosis type 2
Ribhav Pasricha1, Sachin Anil Borkar1, Kanwaljeet Garg1, Leve Joseph2, Bhawani Shankar Sharma1
1 Department of Neurosurgery, All India Institute of Medical Sciences, New Delhi, India
2 Department of Neuroradiology, All India Institute of Medical Sciences, New Delhi, India
|Date of Web Publication||1-Mar-2016|
Dr. Sachin Anil Borkar
Room No 717, 7th Floor, Department of Neurosurgery, Cardio Neuro Center, All India Institute of Medical Sciences, New Delhi
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Pasricha R, Borkar SA, Garg K, Joseph L, Sharma BS. Multiple bilateral cranial nerve schwannomas in a patient with neurofibromatosis type 2. Asian J Neurosurg 2016;11:167-8
|How to cite this URL:|
Pasricha R, Borkar SA, Garg K, Joseph L, Sharma BS. Multiple bilateral cranial nerve schwannomas in a patient with neurofibromatosis type 2. Asian J Neurosurg [serial online] 2016 [cited 2020 Jul 7];11:167-8. Available from: http://www.asianjns.org/text.asp?2016/11/2/167/177634
Neurofibromatosis type 2 (NF2) or multiple inherited schwannomas, meningiomas, and ependymomas syndrome is an autosomal dominant disease characterized by the development of bilateral vestibular schwannomas. We present a rare case of NF2 with multiple cranial nerve schwannomas involving the 3rd, 5th, 6th, 7th, 8th, 11th, and 12th cranial nerves. The patient, in addition, had anterior third right parasagittal meningioma and C2–C4 and C7 neurofibromas. A patient of NF2 with so many bilateral cranial nerve schwannomas has never been described in the literature.,
A 20-year-old female presented with decreased hearing in both ears and decreased vision in her left eye for 2 years. Neurological examination revealed a visual acuity in the left eye of 6/60. There was bilateral sensorineural hearing loss. Magnetic resonance imaging revealed multiple extra axial lobulated masses along the course of bilateral 3rd, 5th, 6th, 8th, 11th, and 12th cranial nerves [Figure 1] and [Figure 2]. Symmetrical masses were seen along the course of both trigeminal nerves involving their cisternal segments [Figure 1]c. Symmetrical lesions were also seen causing widening of both internal auditory canals with cisternal extension. Lobulated masses were also seen along the course of 9th, 10th, and 11th nerves in the jugular foramen. The lesions were showing relatively homogenous signal characteristics, appearing isointense to gray matter on both T1- and T2-fluid attenuation inversion recovery images. There was no significant intralesional hemorrhage or necrosis seen in any of these masses.
|Figure 1: (a) Magnetic resonance imaging constructive interference in steady state images axial sections showing bilateral 3rd nerve schwannoma, (b) magnetic resonance imaging constructive interference in steady state images axial sections showing right 6th nerve schwannoma, (c) postcontrast magnetic resonance imaging images axial sections showing bilateral 5th and 8th nerve schwannoma, (d and e) postcontrast magnetic resonance imaging images axial sections showing intraspinal and extraspinal components of 11th nerve schwannoma, (f) postcontrast magnetic resonance imaging images axial sections showing 12th nerve schwannoma|
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|Figure 2: Postcontrast magnetic resonance imaging images axial sections showing various schwannomas|
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Surgical treatment of patients with NF2 is complex. Management in such patients consists of removal of only symptomatic lesions with regular surveillance for the other lesions. Bilateral vestibular schwannoma may complicate the decision of surgery. The goal of surgery in such cases is to decompress the brain stem and to prolong the period of cranial nerve function. Another alternative is to wait until the affected ear becomes deaf. Larger tumors may require partial removal with decompression performed when brain stem compression develops.
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Conflicts of interest
There are no conflicts of interest.
| References|| |
Parteki S, Raval M, Kumari R, Singhal S, Gupta N. Multiple cranial nerve schwannomas with multifocal cystic meningiomatosis in a case of neurofibromatosis type 2. Eur J Radiol Extra 2010;75:e87-91.
Halefoglu AM. Neurofibromatosis type 2 associated with multiple cranial nerve schwannomas: A case report. Kulak Burun Bogaz Ihtis Derg 2007;17:171-5.
Spilberg G, Marchiori E, Gasparetto EL, Cabral RF, Takayassu TC, Batista RR, et al.
Magnetic resonance findings of neurofibromatosis type 2: A case report. Cases J 2009;2:6720.
[Figure 1], [Figure 2]