Radiographic progression patterns and treatment response in adult H3 K27-altered diffuse midline glioma: a descriptive review and single center case series
5 affiliations
- 1Department of Radiology, Medical College of Wisconsin
- 2Department of Biophysics, Medical College of Wisconsin
- 3Department of Neurology, Medical College of Wisconsin
- 4Department of Pathology, Medical College of Wisconsin
- 5Department of Biomedical Engineering, Medical College of Wisconsin
Abstract
Background: Diffuse midline glioma (DMG) with H3 K27 mutations are aggressive central nervous system tumors with limited treatment options and poor outcomes. Although more common in children, there is increasing recognition of adult cases, which remain under-characterized regarding longitudinal imaging trends and response to multimodal treatment. We aim to highlight heterogeneity and describe patterns of adult H3 K27-altered DMG in cases assessed at autopsy.
Methods: Patients were included in the study if they were ≥ 21 years of age, and had a confirmed diagnosis of DMG, H3 K27-altered. Manual segmentations of contrast enhancement (CE) on T1-weighted post-contrast imaging and hyperintense FLAIR regions were performed at each timepoint. CE ratios (CE vol /FLAIR vol ) were calculated to characterize enhancing versus infiltrative tumor burden. Treatment timelines were retrospectively aligned with imaging timing to evaluate disease progression and therapy response.
Results: This case series included six patients with confirmed H3 K27-altered DMG. Longitudinal imaging data revealed radiographic heterogeneity between patients. Periods of FLAIR reduction were observed to occur around bevacizumab treatment. Mean CE ratios were lowest in Patients 5 and 1 (0.081 and 0.225 respectively), intermediate in Patient 4 (0.312), and highest in Patient 3 (0.668). ATRX-mutant cases ( n , 2) had comparatively short overall survival of 26 and 164 days while ATRX-wild type cases ( n , 4) survived 728-2,299 days.
Conclusions: Among our cohort, we observed two patients with ATRX mutations to have comparatively short survival periods. Findings highlight heterogeneity of adult H3 K27-altered DMG, and limitations of imaging-based modalities to track tumor progression.
Related
- Diabetic Retinal Disease Onset and Progression During Established Semaglutide or Tirzepatide Treatment: A Retrospective Comparative Cohort Study With a Descriptive Regional Nonarteritic Anterior Ischemic Optic Neuropathy (NAION) Review.
- Durable biochemical and radiographic response during toripalimab treatment in MSI-high/TMB-high mCRPC: a case report
- Genetic Predictors of Curve Progression and Brace Treatment Response in Adolescent Idiopathic Scoliosis: Toward Personalized Management
- Functional decline at progression after durvalumab in stage III non-small cell lung cancer: prognostic impact and treatment patterns in a multicenter retrospective cohort study.
- Treatment response biomarkers in early Alzheimers disease: longitudinal trajectories, sample size estimates, and the impact of progression variability
BibTeX
@article{lahrache2026radiographic,
title = {{Radiographic progression patterns and treatment response in adult H3 K27-altered diffuse midline glioma: a descriptive review and single center case series}},
author = {Adam Lahrache and Hope M. Reecher and Aleksandra Winiarz and Savannah R. Duenweg and Daniel Kim and Allison K. Lowman and Biprojit Nath and Benjamin E. Chao and Fitzgerald Kyereme and Michael J. Barrett and Mrina Mtenga and Jennifer Connelly and Daniel Destiche and Mohit Agarwal and Elaine Tanhehco and Jamie Jacobsohn and Samuel A. Bobholz and Peter S. LaViolette},
journal = {Frontiers in Oncology},
year = {2026},
month = oct,
doi = {10.3389/fonc.2026.1934399},
url = {https://www.frontiersin.org/articles/10.3389/fonc.2026.1934399/full},
note = {CC BY 4.0},
}
RIS
TY - JOUR
TI - Radiographic progression patterns and treatment response in adult H3 K27-altered diffuse midline glioma: a descriptive review and single center case series
AU - Adam Lahrache
AU - Hope M. Reecher
AU - Aleksandra Winiarz
AU - Savannah R. Duenweg
AU - Daniel Kim
AU - Allison K. Lowman
AU - Biprojit Nath
AU - Benjamin E. Chao
AU - Fitzgerald Kyereme
AU - Michael J. Barrett
AU - Mrina Mtenga
AU - Jennifer Connelly
AU - Daniel Destiche
AU - Mohit Agarwal
AU - Elaine Tanhehco
AU - Jamie Jacobsohn
AU - Samuel A. Bobholz
AU - Peter S. LaViolette
PY - 2026
DA - 2026/10/26
JO - Frontiers in Oncology
DO - 10.3389/fonc.2026.1934399
UR - https://www.frontiersin.org/articles/10.3389/fonc.2026.1934399/full
AB - Background: Diffuse midline glioma (DMG) with H3 K27 mutations are aggressive central nervous system tumors with limited treatment options and poor outcomes. Although more common in children, there is increasing recognition of adult cases, which remain under-characterized regarding longitudinal imaging trends and response to multimodal treatment. We aim to highlight heterogeneity and describe patterns of adult H3 K27-altered DMG in cases assessed at autopsy. Methods: Patients were included in the study if they were ≥ 21 years of age, and had a confirmed diagnosis of DMG, H3 K27-altered. Manual segmentations of contrast enhancement (CE) on T1-weighted post-contrast imaging and hyperintense FLAIR regions were performed at each timepoint. CE ratios (CE vol /FLAIR vol ) were calculated to characterize enhancing versus infiltrative tumor burden. Treatment timelines were retrospectively aligned with imaging timing to evaluate disease progression and therapy response. Results: This case series included six patients with confirmed H3 K27-altered DMG. Longitudinal imaging data revealed radiographic heterogeneity between patients. Periods of FLAIR reduction were observed to occur around bevacizumab treatment. Mean CE ratios were lowest in Patients 5 and 1 (0.081 and 0.225 respectively), intermediate in Patient 4 (0.312), and highest in Patient 3 (0.668). ATRX-mutant cases ( n , 2) had comparatively short overall survival of 26 and 164 days while ATRX-wild type cases ( n , 4) survived 728-2,299 days. Conclusions: Among our cohort, we observed two patients with ATRX mutations to have comparatively short survival periods. Findings highlight heterogeneity of adult H3 K27-altered DMG, and limitations of imaging-based modalities to track tumor progression.
SN - 2234-943X
ER -