ArticleNeuro-oncology2025
Distinct molecular profile and outcome of oligodendroglioma, IDH-mutant, 1p/19q-codeleted, and TERTp-wildtype: A grade 1 oligodendroglioma of young patients?
Article in Neuro-oncology, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 3 papers.
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3 citing papers in PubMed.
- Meningioma with trabecular architecture: a harbinger of BAP1 deficiency.Brain tumor pathology · 2026Article
- Astrocytes in neuroinflammation and brain cancer.Molecular biomedicine · 2026Review
- Functional analysis of telomere maintenance mechanisms is more informative than immunohistochemistry for ATRX mutation interpretation in Gliomas.Acta neuropathologica communications · 2025Article
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Abstract
backgroundOligodendrogliomas, characterized by isocitrate dehydrogenase (IDH) mutations and 1p/19q codeletion, often exhibit telomerase reverse transcriptase promoter (TERTp) mutations, which have been linked to telomere maintenance (TM) and tumor proliferation. Although there are a few reports on a TERTp-wildtype subset of these tumors in adolescents and young adults, the frequency, molecular characteristics, and prognostic implications of TERTp-wildtype status in oligodendrogliomas remain elusive.
methodsWe retrospectively analyzed 166 IDH-mutant and 1p/19q-codeleted oligodendroglioma cases through comprehensive histopathological review and molecular analyses, including Sanger sequencing, DNA methylation profiling, and whole-exome sequencing (WES).
resultsA TERTp-wildtype status was observed in 20/166 cases (12.0%) and was significantly associated with noticeably young age (age range: 14-27, P < .001), CNS WHO grade 2 (P = .003), and the absence of additional DNA copy number variations (CNVs) beyond the pathognomonic 1p/19q codeletion (P < .001). Epigenetic profiling demonstrated TERTp-wildtype tumors shaped a distinct subgroup at the utmost periphery of TERTp-mutant oligodendrogliomas. Methylation analysis of the upstream and proximal TERTp regions revealed that, in line with the absence of genetic alterations, epigenetic regulation does not favor TERT overexpression in TERTp-wildtype oligodendrogliomas. WES showed no TM-related gene alterations in TERTp-wildtype cases. Cox regression analysis confirmed TERTp-wildtype status as an independent prognostic factor for more favorable progression-free survival (PFS) (P = .009).
conclusionsIn conclusion, "oligodendroglioma, IDH-mutant, 1p/19q-codeleted, and TERTp-wildtype" represent a distinct molecular subgroup associated with younger age and a better clinical course compared to CNS WHO grade 2 oligodendrogliomas.
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