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CC BY 4.0 · J Neurol Surg Rep 2025; 86(02): e57-e64
DOI: 10.1055/a-2547-5320
Review Report

Malignant Transformation of a Vestibular Schwannoma Without Previous Radiation Exposure: Illustrative Case and Literature Review

Chao Li    *
1   Department of Neurosurgery, The University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma
,
James Fowler   *
2   Department of Neurosurgery, Hartford Hospital, Hartford, Connecticut
,
Kishore Balasubramanian
1   Department of Neurosurgery, The University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma
3   Texas A&M Health Science Center, Bryan, Texas
,
Kar-Ming Fung
1   Department of Neurosurgery, The University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma
,
Piao Zhe
4   Department of Pathology, Kaiser Fontana, Fontana, California
,
William W. Wu
4   Department of Pathology, Kaiser Fontana, Fontana, California
› Author Affiliations Funding None.
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Abstract

Background

Although malignant transformation of benign vestibular schwannoma (VS) preceded by irradiation has been well documented, few studies have demonstrated malignant transformation in the absence of radiation. Here, we present a rare case of the malignant transformation of a benign VS to a malignant peripheral nerve sheath tumor (MPNST) in the absence of prior irradiation. Additionally, we conducted a literature search to identify all other reported cases of MPNST arising from VS under similar conditions.

Case Presentation

A 75-year-old female presented to the hospital with a 1-month history of left-sided facial numbness, loss of taste on the left side of her tongue, severe dysarthria, and recent-onset cranial nerve VI and VII palsies. MRI of the brain with and without contrast demonstrated an enlarging cerebellopontine angle mass and signs of brainstem compression. The patient underwent a left retrosigmoid craniotomy and surgical resection. Pathology and immunohistochemistry sequencing findings were consistent for MPNST with rhabdomyoblastic differentiation (malignant triton tumor). An outside review of the case by a large academic institution concurred with the diagnosis. The patient did not report any previous history of irradiation.

Conclusion

A total of 11 cases, including ours, have appropriate S-100 immunochemical reactivity to confirm malignant transformation. Due to the limited number of reported cases of MPNST arising from VS without prior irradiation, information regarding pathogenesis and pathological diagnosis is scarce. We provide valuable additions to the literature, including next-generation sequencing data, to identify potentially targetable genetic changes and help elucidate the pathogenesis of MPNST.

Keywords

vestibular schwannoma (VS) - malignant peripheral nerve sheath tumor (MPNST) - rhabdomyoblastic differentiation - malignant triton tumor - malignant transformation - cerebellopontine angle mass - immunohistochemistry - next-generation sequencing - S-100

Author's Contribution

CL, JF, and KB contributed to the concept of the manuscript and drafted the manuscript. KMF, PZ, and WW revised the manuscript and contributed to the concept of the manuscript. KMF, JF, and CL contributed to the obtainment and interpretation of the clinical information. All authors have read and approved the final version of the manuscript. Dr. Chao Li and Dr. James Fowler contributed equally.


Data Availability

Sequencing data was obtained through a third-party vendor, Strata Oncology, and given as a report. Data was not deposited in a registry due to a lack of access to the primary sequencing data.


Consent for Publication

The patient has consented to submission of this case report for journal publication, and we have obtained written informed consent.


* Authors contributed equally.




Publication History

Received: 17 December 2024

Accepted: 11 February 2025

Article published online:
03 April 2025

© 2025. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution License, permitting unrestricted use, distribution, and reproduction so long as the original work is properly cited. (https://creativecommons.org/licenses/by/4.0/)

Georg Thieme Verlag KG
Oswald-Hesse-Straße 50, 70469 Stuttgart, Germany

 

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