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Published on: November 17, 2021
[Pediatric Glioma]
Keita Terashima1, Hideki Ogiwara
1Division of Neuro-Oncology, Children's Cancer Center, National Center for Child Health and Development.
Insights
Pediatric gliomas are rare brain tumors with diverse types. Molecular advancements reveal genetic targets, offering hope for new BRAF/MEK-inhibitor therapies and novel agents for infant gliomas.
Area of Science:
- Pediatric neuro-oncology
- Molecular neuropathology
- Cancer genetics
Background:
- Pediatric gliomas encompass diverse histopathological subtypes, classified as low- or high-grade.
- Key clinical types include cerebellar astrocytomas, pilocytic astrocytomas, and brainstem gliomas.
- Management requires specialized neurosurgical centers and multimodal treatments like radiotherapy and chemotherapy.
Purpose of the Study:
- To review current understanding of pediatric gliomas, focusing on classification, treatment, and emerging molecular targets.
- To highlight the significance of genetic aberrations in guiding therapeutic strategies for pediatric gliomas.
Main Methods:
- Review of current literature on pediatric glioma classification and treatment.
- Analysis of recent advancements in molecular genetics and targeted therapies.
- Discussion of neurosurgical, oncological, and genetic approaches.
Main Results:
- Low-grade gliomas frequently exhibit RAS/MAPK pathway anomalies (e.g., BRAF-KIAA1549 fusion, BRAF V600E mutation) responsive to BRAF/MEK-inhibitors.
- Diffuse midline gliomas, including diffuse intrinsic pontine gliomas, often harbor H3K27M mutations, lacking targeted agents.
- Infant gliomas are increasingly sub-categorized by genetic abnormalities, with potential for novel agents targeting ALK, ROS1, or NTRK fusions.
Conclusions:
- Pediatric gliomas require specialized care due to their rarity and complexity.
- Molecular profiling is crucial for accurate diagnosis and personalized treatment strategies.
- Targeted therapies based on genetic aberrations show promise for improving outcomes in pediatric gliomas.
Abstract:
Pediatric gliomas include various types of glioma broadly categorized as low- or hi-grade based on histopathological features. Clinically significant types include cerebellar astrocytomas, optic pathway / hypothalamic pilocytic astrocytomas, and brainstem gliomas. Neurosurgical roles vary for different kinds of pediatric gliomas. Since these representative tumors remain rare, the patients should be directed toward facilities with experienced neurosurgeons. Radiotherapy and chemotherapy are very important as either adjuvant or primary treatment modalities. Recent advancements in molecular biology have revealed unique genetic aberrations in different types of pediatric gliomas. The RAS/MAPK pathway anomalies, including BRAF-KIAA1549 fusion and BRAF V600E mutation, are present in most low-grade gliomas. BRAF/MEK-inhibitors have yielded promising clinical study results. Diffuse midline gliomas, including diffuse intrinsic pontine gliomas, often harbor H3 mutations such as H3K27M. Agents that target these molecular aberrations are unavailable. Because gliomas in infants are sub-categorized by their genetic abnormalities, novel agents targeting ALK, ROS1, or NTRK fusions are promising treatments.
