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Temporal Bone Histopathology in Neurofibromatosis Type 2

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Author(s)
Sung-Il NamFred H. Linthicum, Jr.Saumil N. Merchant
Keimyung Author(s)
Nam, Sung Il
Department
Dept. of Otorhinolaryngology (이비인후과학)
Journal Title
Laryngoscope
Issued Date
2011
Volume
121
Issue
7
Keyword
Neurofibromatosis type 2bilateral acoustic neuromatemporal bone histopathologyotopathology
Abstract
Objectives/Hypothesis: To describe the histopathologic findings in the temporal bone in patients with neurofibromato-
sis type 2 (NF2). The literature contains limited data on otopathology of NF2.
Study Design: Basic science study.
Methods: Twenty-six temporal bones from 16 patients with NF2 were examined by light microscopy. The diagnosis of
NF2 was made on the basis of bilateral cochleovestibular schwannomas. Clinical information was obtained from review of the
medical records.
Results: The tumors were multicentric in origin in 19 of 26 (73%) ears. Typically, tumors were seen arising within the
internal auditory canal and from various locations within the labyrinth. The majority of schwannomas showed high cellularity
with involvement of the labyrinth. Most cases showed significant degrees of degeneration of sensory and neural elements
within the cochlea. Fusion tumors were sometimes seen as a result of a schwannoma merging with an adjacent meningioma.
Fifteen of 26 (58%) ears showed facial nerve involvement by schwannoma.
Conclusions: Cochleovestibular schwannomas in NF2 are aggressive neoplasms; they are often multicentric and demon-
strate a propensity to involve the labyrinth. There is often associated secondary degeneration within the cochlea. These fea-
tures make total removal of these tumors and their removal with preservation of hearing more difficult than with sporadic
unilateral cochleovestibular schwannoma.
Key Words: Neurofibromatosis type 2, bilateral acoustic neuroma, temporal bone histopathology, otopathology.
121:1548–1554, 2011
Keimyung Author(s)(Kor)
남성일
Publisher
School of Medicine
Citation
Sung-Il Nam et al. (2011). Temporal Bone Histopathology in Neurofibromatosis Type 2. Laryngoscope, 121(7), 1548–1554. doi: 10.1002/lary.21822
Type
Article
ISSN
0023-852X
DOI
10.1002/lary.21822
URI
https://kumel.medlib.dsmc.or.kr/handle/2015.oak/34637
Appears in Collections:
1. School of Medicine (의과대학) > Dept. of Otorhinolaryngology (이비인후과학)
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