Diffuse brainstem gliomas across the pediatric, adolescent, and adult age spectrum: Clinical and radiological features, molecular profile, and determinants of survival in a multicenter retrospective cohort of 42 patients.
- 1Department of Neurosurgery, Instituto Nacional de Neurología y Neurocirugía "Manuel Velasco Suárez", Mexico City, Mexico.
- 2Department of Radioneurosurgery, Instituto Nacional de Neurología y Neurocirugía "Manuel Velasco Suárez", Mexico City, Mexico.
Abstract
Background
Diffuse brainstem gliomas are infiltrative neoplasms classically described in children as diffuse intrinsic pontine glioma, but increasingly recognized in adults. We describe the clinical, radiological, molecular, and survival characteristics of a cohort spanning the full age spectrum and identify determinants of overall survival (OS).Methods
Retrospective analysis of 42 consecutive patients with a clinical-radiological diagnosis of diffuse brainstem glioma treated between 1996 and 2025 at two neuro-oncology referral centers in Mexico. Demographic, clinical, imaging, biopsy, molecular (histone H3 lysine-27-to-methionine mutation [H3 K27M], isocitrate dehydrogenase 1 [IDH1], O²-methylguanine-DNA methyltransferase [MGMT], codeletion of chromosome arms 1p and 19q [1p/19q], epidermal growth factor receptor [EGFR], and tumour protein 53 gene [TP53]), and treatment variables were analyzed after a database audit. OS was estimated by Kaplan-Meier, compared by log-rank test, and modeled by multivariable Cox regression.Results
Median age at onset was 31.1 years (range 7.8-65.7); 10 patients (23.8%) were younger than 18 years, and 33 (78.6%) were male. Stereotactic biopsy was performed in 32 patients (76.2%). H3 K27M was mutant in 12 (28.6%). Median OS was 23.3 months (95% confidence interval [CI] 14.7-32.5). H3 K27M mutation (hazard ratio [HR] 10.35; 95% CI 3.77-28.40) and contrast enhancement (HR 2.71; 95% CI 1.28-5.71) were independent adverse prognostic factors (concordance index 0.76). A score combining both features separated low-, high-, and very-high-risk groups, with median OS of 36.3, 20.5, and 9.7 months (p < 0.001).Conclusion
H3 K27M status and contrast enhancement were the strongest independent determinants of survival and jointly provide an accessible prognostic score. These findings support systematic molecular characterization, aided by stereotactic biopsy when safely feasible, for prognostic stratification and access to targeted therapies.
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BibTeX
@article{garciabitar2026diffuse,
title = {{Diffuse brainstem gliomas across the pediatric, adolescent, and adult age spectrum: Clinical and radiological features, molecular profile, and determinants of survival in a multicenter retrospective cohort of 42 patients.}},
author = {Andrea Garcia-Bitar and Guillermo Axayacalt Gutierrez-Aceves and Alejandro Rodriguez-Camacho and Jorge Alejandro Torres-Rios and Jose Guillermo Flores-Vazquez and Lilian Zavala-Romero and Gerardo Gomez-Castro and Alejandro Salazar-Pigeon and Sergio Moreno-Jiménez},
journal = {Surgical neurology international},
year = {2026},
month = oct,
doi = {10.25259/sni_964_2026},
url = {https://europepmc.org/article/MED/42974225},
note = {CC BY-NC-SA},
}
RIS
TY - JOUR
TI - Diffuse brainstem gliomas across the pediatric, adolescent, and adult age spectrum: Clinical and radiological features, molecular profile, and determinants of survival in a multicenter retrospective cohort of 42 patients.
AU - Andrea Garcia-Bitar
AU - Guillermo Axayacalt Gutierrez-Aceves
AU - Alejandro Rodriguez-Camacho
AU - Jorge Alejandro Torres-Rios
AU - Jose Guillermo Flores-Vazquez
AU - Lilian Zavala-Romero
AU - Gerardo Gomez-Castro
AU - Alejandro Salazar-Pigeon
AU - Sergio Moreno-Jiménez
PY - 2026
DA - 2026/10/02
JO - Surgical neurology international
DO - 10.25259/sni_964_2026
UR - https://europepmc.org/article/MED/42974225
AB - Background Diffuse brainstem gliomas are infiltrative neoplasms classically described in children as diffuse intrinsic pontine glioma, but increasingly recognized in adults. We describe the clinical, radiological, molecular, and survival characteristics of a cohort spanning the full age spectrum and identify determinants of overall survival (OS).Methods Retrospective analysis of 42 consecutive patients with a clinical-radiological diagnosis of diffuse brainstem glioma treated between 1996 and 2025 at two neuro-oncology referral centers in Mexico. Demographic, clinical, imaging, biopsy, molecular (histone H3 lysine-27-to-methionine mutation [H3 K27M], isocitrate dehydrogenase 1 [IDH1], O²-methylguanine-DNA methyltransferase [MGMT], codeletion of chromosome arms 1p and 19q [1p/19q], epidermal growth factor receptor [EGFR], and tumour protein 53 gene [TP53]), and treatment variables were analyzed after a database audit. OS was estimated by Kaplan-Meier, compared by log-rank test, and modeled by multivariable Cox regression.Results Median age at onset was 31.1 years (range 7.8-65.7); 10 patients (23.8%) were younger than 18 years, and 33 (78.6%) were male. Stereotactic biopsy was performed in 32 patients (76.2%). H3 K27M was mutant in 12 (28.6%). Median OS was 23.3 months (95% confidence interval [CI] 14.7-32.5). H3 K27M mutation (hazard ratio [HR] 10.35; 95% CI 3.77-28.40) and contrast enhancement (HR 2.71; 95% CI 1.28-5.71) were independent adverse prognostic factors (concordance index 0.76). A score combining both features separated low-, high-, and very-high-risk groups, with median OS of 36.3, 20.5, and 9.7 months (p < 0.001).Conclusion H3 K27M status and contrast enhancement were the strongest independent determinants of survival and jointly provide an accessible prognostic score. These findings support systematic molecular characterization, aided by stereotactic biopsy when safely feasible, for prognostic stratification and access to targeted therapies.
SN - 2229-5097
SN - 2152-7806
ER -