Central nervous system schwannoma, VGLL-fused (EWSR1::VGLL1 fusion) with neuroblastoma-like cell dense areas in the frontal lobe of a young man with schwannomatosis due to a germline LZTR1 mutation

Authors

  • David G. Munoz Laboratory Medicine and Pathobiology, University of Toronto, Toronto, ON, Canada and Department of Laboratory Medicine, Unity Health Toronto, Toronto, ON, Canada
  • Sunit Das Department of Surgery, Division of Neurosurgery, Unity Health Toronto, Toronto, ON, Canada
  • Ju-Yoon Yoon Laboratory Medicine and Pathobiology, University of Toronto, Toronto, ON, Canada and Department of Laboratory Medicine, Unity Health Toronto, Toronto, ON, Canada
  • Robert Siddaway Department of Laboratory Medicine, Hospital for Sick Children, Toronto, ON, Canada
  • Adrian Levine Department of Laboratory Medicine, Hospital for Sick Children, Toronto, ON, Canada
  • Kenneth D. Aldape Laboratory of Pathology, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, MD, USA

DOI:

https://doi.org/10.17879/freeneuropathology-2026-8898

Keywords:

EWSR1::VGLL1 fusion, LZTR1 mutation, Central nervous system schwannoma

Abstract

We report a central nervous system schwannoma, VGLL-fused in a young man’s frontal lobe. Somatic abnormalities included an EWSR1::VGLL1 fusion, which incorporated the entire translated region of VGLL1, but excluded most domains of EWSR1. The tumor histologically merged with the brain, and showed both schwannoma-like and neuroblastoma-like areas. A germline LZTR1 mutation was subsequently identified, implying the patient suffered from schwannomatosis.

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Published

2026-01-08

How to Cite

Munoz, D. G., Das, S., Yoon, J.-Y., Siddaway, R., Levine, A., & Aldape, K. D. (2026). Central nervous system schwannoma, VGLL-fused (EWSR1::VGLL1 fusion) with neuroblastoma-like cell dense areas in the frontal lobe of a young man with schwannomatosis due to a germline LZTR1 mutation. Free Neuropathology, 7, 1. https://doi.org/10.17879/freeneuropathology-2026-8898

Issue

Vol. 7 (2026)

Section

Case Reports

License

Copyright (c) 2026 David G. Munoz, Sunit Das, Ju-Yoon Yoon, Robert Siddaway, Adrian Levine, Kenneth D. Aldape

Creative Commons License

This work is licensed under a Creative Commons Attribution 4.0 International License.

Papers are published open access under the Creative Commons BY 4.0 license. This license lets others distribute, remix, adapt, and build upon your work, even commercially, as long as they credit you for the original creation. Data included in the article are made available under the CC0 1.0 Public Domain Dedication waiver, unless otherwise stated, meaning that all copyrights are waived.