Multiple Unilateral Vestibular Schwannomas: Segmental NF2 or Sporadic Occurrence?
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. Setti...
| Main Authors: | , |
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| Format: | Article |
| Language: | English |
| Published: |
Georg Thieme Verlag KG
2016-06-01
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| Series: | Journal of Neurological Surgery Reports |
| Subjects: | |
| Online Access: | http://www.thieme-connect.de/DOI/DOI?10.1055/s-0036-1584603 |
| _version_ | 1811227405344309248 |
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| author | Matthew L. Carlson Jamie J. Van Gompel |
| author_facet | Matthew L. Carlson Jamie J. Van Gompel |
| author_sort | Matthew L. Carlson |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-04-12T09:41:59Z |
| format | Article |
| id | doaj.art-28cfb70912c0462083043bd15fcd81ba |
| institution | Directory Open Access Journal |
| issn | 2193-6358 2193-6366 |
| language | English |
| last_indexed | 2024-04-12T09:41:59Z |
| publishDate | 2016-06-01 |
| publisher | Georg Thieme Verlag KG |
| record_format | Article |
| series | Journal of Neurological Surgery Reports |
| spelling | doaj.art-28cfb70912c0462083043bd15fcd81ba2022-12-22T03:38:03ZengGeorg Thieme Verlag KGJournal of Neurological Surgery Reports2193-63582193-63662016-06-017702e106e10810.1055/s-0036-1584603Multiple Unilateral Vestibular Schwannomas: Segmental NF2 or Sporadic Occurrence?Matthew L. Carlson0Jamie J. Van Gompel1Department of Otolaryngology-Head and Neck Surgery, Mayo Clinic, Rochester, Minnesota, United StatesDepartment of Otolaryngology-Head and Neck Surgery, Mayo Clinic, Rochester, Minnesota, United StatesAbstract 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.http://www.thieme-connect.de/DOI/DOI?10.1055/s-0036-1584603vestibular schwannomainternal auditory canalmagnetic resonance imagingneurofibromatosis type 2 |
| spellingShingle | Matthew L. Carlson Jamie J. Van Gompel Multiple Unilateral Vestibular Schwannomas: Segmental NF2 or Sporadic Occurrence? Journal of Neurological Surgery Reports vestibular schwannoma internal auditory canal magnetic resonance imaging neurofibromatosis type 2 |
| title | Multiple Unilateral Vestibular Schwannomas: Segmental NF2 or Sporadic Occurrence? |
| title_full | Multiple Unilateral Vestibular Schwannomas: Segmental NF2 or Sporadic Occurrence? |
| title_fullStr | Multiple Unilateral Vestibular Schwannomas: Segmental NF2 or Sporadic Occurrence? |
| title_full_unstemmed | Multiple Unilateral Vestibular Schwannomas: Segmental NF2 or Sporadic Occurrence? |
| title_short | Multiple Unilateral Vestibular Schwannomas: Segmental NF2 or Sporadic Occurrence? |
| title_sort | multiple unilateral vestibular schwannomas segmental nf2 or sporadic occurrence |
| topic | vestibular schwannoma internal auditory canal magnetic resonance imaging neurofibromatosis type 2 |
| url | http://www.thieme-connect.de/DOI/DOI?10.1055/s-0036-1584603 |
| work_keys_str_mv | AT matthewlcarlson multipleunilateralvestibularschwannomassegmentalnf2orsporadicoccurrence AT jamiejvangompel multipleunilateralvestibularschwannomassegmentalnf2orsporadicoccurrence |