ArticleNeuro-oncology2022
Characteristics of patients ≥10 years of age with diffuse intrinsic pontine glioma: a report from the International DIPG/DMG Registry.
Article in Neuro-oncology, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 17 papers.
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Who cites it
17 citing papers in PubMed, 22 citations in OpenAlex.
- Adult Thalamic Diffuse Midline Glioma, H3 K27-Altered, WithJournal of medical cases · 2026Article
- Radiological complete response in diffuse intrinsic pontine glioma: a case report.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2026Article
- Epigenetic mechanisms and therapeutic advances in diffuse midline glioma (Review).Oncology reports · 2026Review
- Article
- Frequency and outcomes of midline gliomas in a tertiary care hospital in Pakistan: a retrospective study.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2025Article
- Ophthalmic Presentation of Diffuse Intrinsic Pontine Glioma in Children (Case Series and Literature Review).Neuro-ophthalmology (Aeolus Press) · 2025Article
- Pediatric diffuse intrinsic pontine gliomas- a prospective observational study from a tertiary care neurosurgical center.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2024Observational
- Developmental origins shape the paediatric cancer genome.Nature reviews. Cancer · 2024Review
- ONC201 (Dordaviprone) in Recurrent H3 K27M-Mutant Diffuse Midline Glioma.Journal of clinical oncology : official journal of the American Society of Clinical Oncology · 2024Article
- Review
- Advances in Treatment of Diffuse Midline Gliomas.Current neurology and neuroscience reports · 2023Review
- The cell-cycle choreography of H3 variants shapes the genome.Molecular cell · 2023Review
- Clinical features and outcomes of pediatric intracranial gliomas: results from single center's 226 cases and corroborated with SEER database.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2023Article
- Review
- The current state of glioma data registries.Neuro-oncology advancesArticle
- Treatment-related survival patterns in diffuse intrinsic pontine glioma using a historical cohort: A report from the European Society for Pediatric Oncology DIPG/DMG Registry.Neuro-oncology advancesArticle
- Systematic review of diffuse hemispheric glioma, H3 G34-mutant: Outcomes and associated clinical factors.Neuro-oncology advancesArticle
Corrections and comments
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Authors and funding
51 authors at 20 institutions in 8 countries.
Funding
Abstract
backgroundDiffuse intrinsic pontine gliomas (DIPG) generally occur in young school-age children, although can occur in adolescents and young adults. The purpose of this study was to describe clinical, radiological, pathologic, and molecular characteristics in patients ≥10 years of age with DIPG enrolled in the International DIPG Registry (IDIPGR).
methodsPatients ≥10 years of age at diagnosis enrolled in the IDIPGR with imaging confirmed DIPG diagnosis were included. The primary outcome was overall survival (OS) categorized as long-term survivors (LTS) (≥24 months) or short-term survivors (STS) (<24 months).
resultsAmong 1010 patients, 208 (21%) were ≥10 years of age at diagnosis; 152 were eligible with a median age of 12 years (range 10-26.8). Median OS was 13 (2-82) months. The 1-, 3-, and 5-year OS was 59.2%, 5.3%, and 3.3%, respectively. The 18/152 (11.8%) LTS were more likely to be older (P < .01) and present with longer symptom duration (P < .01). Biopsy and/or autopsy were performed in 50 (33%) patients; 77%, 61%, 33%, and 6% of patients tested had H3K27M (H3F3A or HIST1H3B), TP53, ATRX, and ACVR1 mutations/genome alterations, respectively. Two of 18 patients with IDH1 testing were IDH1-mutant and 1 was a LTS. The presence or absence of H3 alterations did not affect survival.
conclusionPatients ≥10 years old with DIPG have a median survival of 13 months. LTS present with longer symptom duration and are likely to be older at presentation compared to STS. ATRX mutation rates were higher in this population than the general DIPG population.
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