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J Neurol Surg Rep 2016; 77(02): e106-e108
DOI: 10.1055/s-0036-1584603
Case Report
Georg Thieme Verlag KG Stuttgart · New York

Multiple Unilateral Vestibular Schwannomas: Segmental NF2 or Sporadic Occurrence?

Matthew L. Carlson
1   Department of Otolaryngology-Head and Neck Surgery, Mayo Clinic, Rochester, Minnesota, United States
2   Department of Neurologic Surgery, Mayo Clinic, Rochester, Minnesota, United States
,
Jamie J. Van Gompel
1   Department of Otolaryngology-Head and Neck Surgery, Mayo Clinic, Rochester, Minnesota, United States
2   Department of Neurologic Surgery, Mayo Clinic, Rochester, Minnesota, United States
› Author Affiliations
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Publication History

14 February 2016

09 May 2016

Publication Date:
27 June 2016 (online)

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Abstract

Objective To report a case of a patient presenting with two separate unilateral vestibular schwannomas (VSs) without other stigmata of neurofibromatosis type 2 (NF2).

Study Design This article discusses a case report and review of the literature.

Setting Tertiary academic referral center.

Participants A 41-year-old female was referred for evaluation of a left-sided 1.8-cm cerebellopontine angle tumor centered on the porus acusticus and a separate ipsilateral 3-mm intracanalicular tumor appearing to arise from the superior vestibular nerve. The patient denied a family history of NF2. Neurotologic examination was unremarkable and close review of magnetic resonance imaging did not find any other stigmata of NF2.

Results The patient underwent left-sided retrosigmoid craniotomy with gross total resection of both tumors. Final pathology confirmed benign schwannoma. The INI1/SMARCB1 staining pattern did not suggest NF2 or schwannomatosis.

Conclusions This is only the third report of a case with multiple unilateral VSs occurring in a patient without other features of NF2. Herein, the authors review the two other reports and discuss potential mechanisms for this rare phenomenon.

Keywords

vestibular schwannoma - internal auditory canal - magnetic resonance imaging - neurofibromatosis type 2
 

  • References

  • 1 Stivaros SM, Stemmer-Rachamimov AO, Alston R , et al. Multiple synchronous sites of origin of vestibular schwannomas in neurofibromatosis type 2. J Med Genet 2015; 52 (8) 557-562
    CrossrefPubMedSearch in Google Scholar
  • 2 Kennedy RJ, Salzman KL, Shelton C. Unilateral double vestibular schwannoma. Otol Neurotol 2005; 26 (6) 1241-1242
    CrossrefPubMedSearch in Google Scholar
  • 3 Barbara M, Ronchetti F, Manni V, Monini S. Double localization of a unilateral sporadic vestibular schwannoma. Acta Otorhinolaryngol Ital 2008; 28 (1) 34-37
    PubMedSearch in Google Scholar
  • 4 Patil S, Perry A, Maccollin M , et al. Immunohistochemical analysis supports a role for INI1/SMARCB1 in hereditary forms of schwannomas, but not in solitary, sporadic schwannomas. Brain Pathol 2008; 18 (4) 517-519
    PubMedSearch in Google Scholar
  • 5 Evans DG, Lye R, Neary W , et al. Probability of bilateral disease in people presenting with a unilateral vestibular schwannoma. J Neurol Neurosurg Psychiatry 1999; 66 (6) 764-767
    CrossrefPubMedSearch in Google Scholar
  • 6 Luetje CM, Whittaker CK, Callaway LA, Veraga G. Histological acoustic tumor involvement of the VIIth nerve and multicentric origin in the VIIIth nerve. Laryngoscope 1983; 93 (9) 1133-1139
    PubMedSearch in Google Scholar