An Official publication of The Asian Congress of Neurological Surgeons (AsianCNS)

Search Article
Home About us Editorial board Search Ahead of print Current issue Archives Submit article Instructions Advertise Subscribe Contacts Login  Facebook Tweeter
  Users Online: 806 Home Print this page Email this page Small font sizeDefault font sizeIncrease font size  

   Table of Contents      
LETTER TO EDITOR
Year : 2016  |  Volume : 11  |  Issue : 2  |  Page : 167-168

Multiple bilateral cranial nerve schwannomas in a patient with neurofibromatosis type 2


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 Publication1-Mar-2016

Correspondence Address:
Dr. Sachin Anil Borkar
Room No 717, 7th Floor, Department of Neurosurgery, Cardio Neuro Center, All India Institute of Medical Sciences, New Delhi
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/1793-5482.177634

Rights and Permissions

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 Aug 5];11:167-8. Available from: http://www.asianjns.org/text.asp?2016/11/2/167/177634

Sir,

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.[1] 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.[2],[3]

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

Click here to view
Figure 2: Postcontrast magnetic resonance imaging images axial sections showing various schwannomas

Click here to view


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.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
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.  Back to cited text no. 1
    
2.
Halefoglu AM. Neurofibromatosis type 2 associated with multiple cranial nerve schwannomas: A case report. Kulak Burun Bogaz Ihtis Derg 2007;17:171-5.  Back to cited text no. 2
    
3.
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.  Back to cited text no. 3
    


    Figures

  [Figure 1], [Figure 2]


This article has been cited by
1 MISME syndrome: A very rare constellation of multiple supratentorial, infratentorial and multiple spinal tumors in neurofibromatosis type 2
Anil Kumar
Interdisciplinary Neurosurgery. 2019; : 100579
[Pubmed] | [DOI]



 

Top
 
 
  Search
 
<
Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
Access Statistics
Email Alert *
Add to My List *
* Registration required (free)  

 
  In this article
   References
   Article Figures

 Article Access Statistics
    Viewed907    
    Printed10    
    Emailed0    
    PDF Downloaded119    
    Comments [Add]    
    Cited by others 1    

Recommend this journal