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Case Reports
. 2022 Mar 22;14(3):e23381.
doi: 10.7759/cureus.23381. eCollection 2022 Mar.

Schwannomatosis Presenting With a Grade IV Glioblastoma: A Case Report and Literature Review

Nardine Abdelsayed  1 , Zachary Bondranko  1 , Peter Montesano  1
Affiliations
Case Reports

Schwannomatosis Presenting With a Grade IV Glioblastoma: A Case Report and Literature Review

Nardine Abdelsayed et al. Cureus. .

Abstract

Schwannomatosis is a rare subset of neurofibromatosis. It is a disease process with a predisposition to schwannomas in the absence of bilateral vestibular schwannomas, which differentiates it from neurofibromatosis 2 (NF2). It is occasionally associated with certain tumors such as malignant peripheral nerve sheath tumors or rhabdoid tumors. Currently, there is limited literature to suggest an association between schwannomatosis and glioblastoma (GB). We present a case of a 55-year-old female with a history significant for schwannomatosis who presented after a witnessed first-time seizure with left facial weakness and slurred speech. She was found to have a 3 cm right-sided ring-enhancing lesion that was excised and found to be a grade IV Isocitrate dehydrogenase (IDH) wildtype GB.

Keywords: grade iv glioblastoma; lztr1; neurofibromatosis; schwannomatosis; smarcb1.

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Conflict of interest statement

This research was supported (in whole or in part) by HCA and/or an HCA affiliated entity. The views expressed in this publication represent those of the author(s) and do not necessarily represent the official views of HCA or any of its affiliated entities

Figures

Figure 1
Figure 1. Computed tomography (CT) head with the yellow arrow showing 3.0 X 2.9 cm mass in the right cerebral hemisphere near the gray-white junction.
Figure 2
Figure 2. Coronal view on MRI flair with the yellow arrow showing posterior right frontal lobe mass.
Figure 3
Figure 3. 100x, Immunohistochemical analysis for GFAP is positive, consistent with a glial neoplasm.
Figure 4
Figure 4. 100x, Hematoxylin and eosin (H&E) stain showing hyper-cellular glial neoplasm with marked nuclear atypia, geographic necrosis, and microvascular proliferation; consistent with glioblastoma.
Figure 5
Figure 5. Negative immunohistochemical analysis for IDH1 R132H (no nuclear expression), consistent with a wild-type (“de novo”) glioblastoma (100x).
See this image and copyright information in PMC

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