Patterns of care in adult histone mutant gliomas: Results of an international survey

Alexander Yuile; Mustafa Khasraw; Justin T Low; Kyle M Walsh; Eric Lipp; Joanne Sy; Laveniya Satgunaseelan; Marina Ann Kastelan; Madhawa De Silva; Adrian Lee; Helen Wheeler

doi:10.1093/nop/npac047

Patterns of care in adult histone mutant gliomas: Results of an international survey

Neurooncol Pract. 2022 Jun 5;9(6):520-525. doi: 10.1093/nop/npac047. eCollection 2022 Dec.

Authors

Affiliations

¹ Medical Oncology Department, Royal North Shore Hospital, Sydney, Australia.
² The Preston Robert Tisch Brain Tumor Center, Duke Cancer Institute, Duke University, Durham, USA.
³ Department of Neuropathology, Royal Prince Alfred Hospital, Sydney, Australia.
⁴ The Brain Cancer Group, North Shore Private Hospital, Sydney, Australia.

Abstract

Background: Histone mutant gliomas (HMG) with histone H3 K27 and G34 mutations are recognized as biologically discrete entities with distinct anatomical locations, younger age at presentation (in comparison to the most common high-grade gliomas, IDH wildtype glioblastoma), and poor prognosis. There is a paucity of data regarding the management of adult HMG patients and no consensus on management. This study aims to identify current patterns of Australian and US neuro-oncology clinical practice for this entity.

Methods: Following institutional approvals, patterns of care questionnaire designed to capture relevant clinical variables was circulated through the Cooperative Trials Group for Neuro-Oncology (COGNO) in Australia and the Caris Precision Oncology Alliance in the United States (US).

Results: Between 4/2021 and 10/2021, 43 responses were collected. 33% (n = 14) of responders tested all patients for HMGs routinely; 40.92% (n = 18) tested in select patients 26% (n = 11) did not test for HMGs. The common indications for testing selected patients were midline anatomic location (n = 18) and age (n = 11) (<50 years). 23 used molecular sequencing, 22 used IHC at their centers. Nine participants stated knowledge of histone H3 mutations did not affect their management of these gliomas, 11 said it affected their management at the time of recurrence, 23 stated it affected the management of midline K27M patients, 11 participants stated it affected the management of K27M mutant gliomas in other locations, and 3 felt it affected the management of G34R/V mutant gliomas.

Conclusion: Here we present a description of how the discovery of a new molecular subtype of primary glial tumors, histone mutated gliomas in adults, is being introduced into clinical practice.

Keywords: G34; K27M; adult histone mutant glioma; diffuse midline glioma; patterns of care survey.

© The Author(s) 2022. Published by Oxford University Press on behalf of the Society for Neuro-Oncology and the European Association of Neuro-Oncology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.