ArticleNeuro-oncology2018
Diffuse high-grade gliomas with H3 K27M mutations carry a dismal prognosis independent of tumor location.
Article in Neuro-oncology, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 157 papers, 4 of them syntheses that pooled it.
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Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
157 citing papers in PubMed, 4 syntheses or guidelines pooled it.
- Machine learning in neuroimaging for predicting H3K27M mutations in diffuse midline gliomas: a systematic review and meta-analysis.Frontiers in medicine · 2026Pooled it
- Diffuse Midline H3K27-Altered Gliomas in the Spinal Cord: A Systematic Review.Journal of neuro-oncology · 2024Pooled it
- Prognostic Implication of Patient Age in H3K27M-Mutant Midline Gliomas.Frontiers in oncology · 2022Pooled it
- Thalamic gliomas in adults: a systematic review of clinical characteristics, treatment strategies, and survival outcomes.Journal of neuro-oncology · 2021Pooled it
- Automated Determination of the H3 K27-Altered Status in Spinal Cord Diffuse Midline Glioma by Radiomics Based on T2-Weighted MR Images.AJNR. American journal of neuroradiology · 2023Trial
- Entrectinib in children and young adults with solid or primary CNS tumors harboring NTRK, ROS1, or ALK aberrations (STARTRK-NG).Neuro-oncology · 2022Trial
- Mass cytometry detects H3.3K27M-specific vaccine responses in diffuse midline glioma.The Journal of clinical investigation · 2020Trial
- A phase I/II study of ribociclib following radiation therapy in children with newly diagnosed diffuse intrinsic pontine glioma (DIPG).Journal of neuro-oncology · 2020Trial
- Surgical treatment and survival outcome of patients with adult thalamic glioma: a single institution experience of 8 years.Journal of neuro-oncology · 2020Trial
- Molecular, Pathological, Radiological, and Immune Profiling of Non-brainstem Pediatric High-Grade Glioma from the HERBY Phase II Randomized Trial.Cancer cell · 2018Trial
- Clinical and molecular characteristics of thalamic gliomas in adults: prognostic impact of TERT promoter mutation.Neurosurgical review · 2026Article
- Grade-Dependent Prognostic Value of Classical Clinicopathological Factors in Glioma: A Single-Center Retrospective Study.Journal of clinical medicine · 2026Article
- Cerebrospinal Fluid in Pediatric Neuro-Oncology: Molecular Diagnosis, Disease Monitoring, and Clinical Translation.International journal of molecular sciences · 2026Review
- Survival analysis of adult midline-located gliomas and diffuse midline gliomas: beyond the H3K27 mutation paradigm.Journal of neuro-oncology · 2026Article
- Co-occurrence of pediatric-type diffuse high-grade gliomas with arteriovenous malformations: case report and narrative literature review.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2026Review
- Precision diagnosis and therapy for pediatric central nervous system tumors: consensus from the Brazilian Society of Pediatric Oncology (SOBOPE).Lancet regional health. Americas · 2026Review
- Proteogenomics: decoding cancer in multiple dimensions.MedScience · 2026Review
- Early distant progression in adult Histone-3 K27-altered diffuse midline gliomas.Journal of neuro-oncology · 2026Article
- Radiomics and clinical data predict pseudoprogression after radiotherapy in high-grade glioma.Frontiers in oncology · 2026Article
- Case Report: A case of diffuse midline glioma, H3 K27-altered presenting with long-segment spinal cord lesions.Frontiers in oncology · 2026Article
97 more citing papers are in PubMed but not listed here.
Corrections and comments
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Authors and funding
19 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Background: The novel entity of "diffuse midline glioma, H3 K27M-mutant" has been defined in the 2016 revision of the World Health Organization (WHO) classification of tumors of the central nervous system (CNS). Tumors of this entity arise in CNS midline structures of predominantly pediatric patients and are associated with an overall dismal prognosis. They are defined by K27M mutations in H3F3A or HIST1H3B/C, encoding for histone 3 variants H3.3 and H3.1, respectively, which are considered hallmark events driving gliomagenesis. Methods: Here, we characterized 85 centrally reviewed diffuse gliomas on midline locations enrolled in the nationwide pediatric German HIT-HGG registry regarding tumor site, histone 3 mutational status, WHO grade, age, sex, and extent of tumor resection. Results: We found 56 H3.3 K27M-mutant tumors (66%), 6 H3.1 K27M-mutant tumors (7%), and 23 H3-wildtype tumors (27%). H3 K27M-mutant gliomas shared an aggressive clinical course independent of their anatomic location. Multivariate regression analysis confirmed the significant impact of the H3 K27M mutation as the only independent parameter predictive of overall survival (P = 0.009). In H3 K27M-mutant tumors, neither anatomic midline location nor histopathological grading nor extent of tumor resection had an influence on survival. Conclusion: These results substantiate the clinical significance of considering diffuse midline glioma, H3 K27M-mutant, as a distinct entity corresponding to WHO grade IV, carrying a universally fatal prognosis.
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