Pediatric High-Grade Glioma Diagnosis and Treatment
Summary
Pediatric high-grade gliomas (pHGGs) are highly malignant brain tumours in children, characterised by rapid progression and dismal outcomes despite multimodal therapy. Accurate diagnosis integrates clinical presentation—often raised intracranial pressure or focal neurological deficits—with advanced neuroimaging, histopathology and molecular profiling. Modern classification stratifies pHGGs into subtypes such as H3K27-altered diffuse midline glioma, H3G34-mutant hemispheric glioma and H3-wildtype/IDH-wildtype tumours. Surgical resection remains the cornerstone of management where feasible, followed by focal radiotherapy and concomitant chemotherapy. Standard chemotherapeutic agents include temozolomide, although paediatric tumours exhibit distinct biology compared to adult forms, limiting extrapolation of adult protocols. Emerging diagnostic approaches employ DNA methylation profiling and next-generation sequencing to refine molecular subgroups, guiding risk stratification and trial eligibility. Therapeutic advances are centred on targeted agents—such as kinase inhibitors and epigenetic modulators—alongside immunotherapeutic platforms including CAR T cell therapy and peptide vaccines. Clinical trials increasingly incorporate biomarkers of telomere maintenance and tumour microenvironment to improve response prediction. Ongoing research aims to overcome intratumour heterogeneity and therapeutic resistance by integrating single-cell multi-omic analyses, with the ultimate goal of personalising therapy and improving long-term survival.
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Pediatric High-Grade Glioma Diagnosis and Treatment publication trend
The graph below shows the total number of articles in pediatric high-grade glioma diagnosis and treatment across all publications each year (not limited to Nature Index journals).
Technical terms
Diffuse midline glioma: A high-grade glioma with H3K27-alteration arising in midline structures, associated with poor prognosis.
H3G34-mutant: A subtype of hemispheric pHGG marked by mutation at glycine 34 of histone H3, influencing chromatin regulation.
DNA methylation profiling: Genome-wide analysis of methylation patterns used to classify tumour subtypes and predict outcomes.
CAR T cell therapy: Immunotherapy in which patient T cells are engineered to target specific tumour antigens.
Telomere maintenance mechanism: Cellular processes—telomerase activation or alternative lengthening—by which tumours preserve telomere length, affecting prognosis.
References
- Gliomatosis cerebri in children: A poor prognostic phenotype of diffuse gliomas with a distinct molecular profile. Neuro-Oncology (2024).
- Novel insights on genetics and epigenetics as clinical targets for paediatric astrocytoma. Clinical and Translational Medicine (2024).
- Emerging and Biological Concepts in Pediatric High-Grade Gliomas. Cells (2024).
- Applying single cell multi-omic analyses to understand treatment resistance in pediatric high grade glioma. Frontiers in Pharmacology (2023).
- Vorinostat, temozolomide or bevacizumab with irradiation and maintenance BEV/TMZ in pediatric high-grade glioma: A Children’s Oncology Group Study. Neuro-Oncology Advances (2024).
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