CASE REPORT article
Front. Oncol.
Sec. Cancer Molecular Targets and Therapeutics
Volume 15 - 2025 | doi: 10.3389/fonc.2025.1587752
This article is part of the Research TopicInnovative Drug Combinations for Enhanced Solid Tumor Treatment EfficacyView all 7 articles
Advanced Grade 2 Meningioma with PBRM1 Inactivation with Prolonged Response to Immunotherapy
Provisionally accepted
John L Villano1*
Ellen M Reusch1Keng Hee Peh1Rachael M Morgan1Harry D Momo1
David Orren1Stephanie Rock2Thomas Pittman1Janna H Neltner1Jill M Kolesar1- 1University of Kentucky, Lexington, United States
- 2Caris Life Sciences Inc., Irving, Texas, United States
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Meningiomas are the most common primary tumor in the central nervous system, yet an effective systemic treatment remains a challenge. We present a grade 2 meningioma resulting in a positive and prolonged response with pembrolizumab. Our case had PBRM1 and BAP1 functional loss, tumor mutational burden of 4 Muts/Mb, stable microsatellite status, and a PD-L1 tumor proportional score of <1%. We add to the limited literature regarding PBRM1 mutations in meningiomas. We discuss our findings in relation to the ongoing investigation of immune checkpoint inhibitors therapy in treating higher-grade refractory meningiomas.
Keywords: Atypical meningioma, Immunotherapy, PBRM1 Grade 2II Meningioma Responding to Immunotherapy, Meningioma, PD- 1/L1
Received: 04 Mar 2025; Accepted: 04 Aug 2025.
Copyright: © 2025 Villano, Reusch, Peh, Morgan, Momo, Orren, Rock, Pittman, Neltner and Kolesar. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
* Correspondence: John L Villano, University of Kentucky, Lexington, United States
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