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
Further Information

Publication History

14 February 2016

09 May 2016

Publication Date:
27 June 2016 (online)

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.