ArticleNeuro-oncology2023
Multi-institutional study of the frequency, genomic landscape, and outcome of IDH-mutant glioma in pediatrics.
Article in Neuro-oncology, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 24 papers.
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Who cites it
24 citing papers in PubMed, 30 citations in OpenAlex.
- Advances in the management of pediatric low-grade glioma.Neurotherapeutics : the journal of the American Society for Experimental NeuroTherapeutics · 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
- Pediatric diffuse low-grade glioma with oligodendroglioma-like features: A case report.Experimental and therapeutic medicine · 2025Article
- Rare IDH hotspot mutations in dysembryoplastic neuroepithelial tumors expand the spectrum of IDH-altered CNS tumors.Acta neuropathologica · 2025Article
- FDA Approval Summary: Vorasidenib for IDH-Mutant Grade 2 Astrocytoma or Oligodendroglioma Following Surgery.Clinical cancer research : an official journal of the American Association for Cancer Research · 2025Article
- Severe Chemoradiotherapy Toxicity in a Pediatric Patient with Leigh Syndrome and Grade IV Isocitrate Dehydrogenase-Mutant Astrocytoma: A Case Report.The American journal of case reports · 2025Article
- Overcoming the blood-brain barrier (BBB) in pediatric CNS tumors: immunotherapy and nanomedicine-driven strategies.Medical oncology (Northwood, London, England) · 2025Review
- A role for early surgical intervention in childhood low-grade glioma? Illustrative case.Journal of neurosurgery. Case lessons · 2025Article
- An atypical presentation of infiltrative diffuse low-grade glioma in an adolescent: case report.BMC neurology · 2025Article
- A population-based analysis of the molecular landscape of glioma in adolescents and young adults reveals insights into gliomagenesis.Nature cancer · 2025Article
- Targeted therapy for pediatric glioma: RAF(t)ing in the molecular era.World journal of pediatrics : WJP · 2025Review
- Central nervous system tumors in adolescents and young adults: A Society for Neuro-Oncology Consensus Review on diagnosis, management, and future directions.Neuro-oncology · 2025Review
- IDH mutant high-grade gliomas.Frontiers in molecular neuroscience · 2025Review
- A comparative analysis of IDH-mutant glioma in pediatric, young adult, and older adult patients.Neuro-oncology · 2024Article
- Treatment Options for IDH-Mutant Malignant Gliomas.Current treatment options in oncology · 2024Review
- Gliomatosis cerebri in children: A poor prognostic phenotype of diffuse gliomas with a distinct molecular profile.Neuro-oncology · 2024Article
- Primary mismatch repair-deficient IDH-mutant astrocytoma in child: Unusual entity.International journal of surgery case reports · 2024Article
- Review
- Advances in pediatric gliomas: from molecular characterization to personalized treatments.European journal of pediatrics · 2024Review
- Pediatric low-grade glioma: State-of-the-art and ongoing challenges.Neuro-oncology · 2024Review
Corrections and comments
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Authors and funding
32 authors at 14 institutions in 1 country.
Funding
Abstract
backgroundThe incidence and biology of IDH1/2 mutations in pediatric gliomas are unclear. Notably, current treatment approaches by pediatric and adult providers vary significantly. We describe the frequency and clinical outcomes of IDH1/2-mutant gliomas in pediatrics.
methodsWe performed a multi-institutional analysis of the frequency of pediatric IDH1/2-mutant gliomas, identified by next-generation sequencing (NGS). In parallel, we retrospectively reviewed pediatric IDH1/2-mutant gliomas, analyzing clinico-genomic features, treatment approaches, and outcomes.
resultsIncidence: Among 851 patients with pediatric glioma who underwent NGS, we identified 78 with IDH1/2 mutations. Among patients 0-9 and 10-21 years old, 2/378 (0.5%) and 76/473 (16.1%) had IDH1/2-mutant tumors, respectively. Frequency of IDH mutations was similar between low-grade glioma (52/570, 9.1%) and high-grade glioma (25/277, 9.0%). Four tumors were graded as intermediate histologically, with one IDH1 mutation. Outcome: Seventy-six patients with IDH1/2-mutant glioma had outcome data available. Eighty-four percent of patients with low-grade glioma (LGG) were managed observantly without additional therapy. For low-grade astrocytoma, 5-year progression-free survival (PFS) was 42.9% (95%CI:20.3-63.8) and, despite excellent short-term overall survival (OS), numerous disease-related deaths after year 10 were reported. Patients with high-grade astrocytoma had a 5-year PFS/OS of 36.8% (95%CI:8.8-66.4) and 84% (95%CI:50.1-95.6), respectively. Patients with oligodendroglioma had excellent OS.
conclusionsA subset of pediatric gliomas is driven by IDH1/2 mutations, with a higher rate among adolescents. The majority of patients underwent upfront observant management without adjuvant therapy. Findings suggest that the natural history of pediatric IDH1/2-mutant glioma may be similar to that of adults, though additional studies are needed.
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