Circumscribed Gliomas

C71WHO Vol. 6 (CNS5, 2021)

Central Nervous System (CNS)

Prognosis

This section is being prepared by our editorial process.

🔬 Histological Types

📚 Latest Research

2026-09-10

Efficacy and safety of trametinib use in pediatric brain tumors in a low- to middle-income country.

Farah R, et al

A retrospective case series from Lebanon's LAU Medical Center Rizk Hospital found that trametinib, a MEK inhibitor, produced clinical improvement in all four pediatric patients with progressive low-grade gliomas (LGGs) harboring distinct molecular drivers (KIAA1549-BRAF, NF1 Loss, and BRAF V600E), with one patient also achieving a partial radiographic response over a median treatment duration of 30.2 months (range 22–35.5 months). The drug was obtained on a compassionate-use basis through an international Managed Access Program, representing the first published report on targeted therapy use in Lebanon and one of very few from low- to middle-income countries (LMICs). Side effects were manageable, consisting primarily of skin reactions and gastrointestinal complaints with only rare therapy interruptions, while quality-of-life assessment using the Pediatric Quality of Life Inventory 4.0 revealed that physical and school functioning were the most affected domains. These findings suggest that MEK inhibitors such as trametinib may be a feasible treatment option for selected pediatric LGG patients in LMIC settings where appropriate diagnostic and monitoring infrastructure is in place.

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery

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2026-09-09

Systemic metastases in a BRAF V600E-mutant pleomorphic xanthoastrocytoma classified by DNA methylation: treatment with BRAF/MEK inhibitors and liquid biopsy monitoring.

Raschio B, et al

A case of BRAF V600E-mutant pleomorphic xanthoastrocytoma (PXA) with rare systemic metastases was accurately classified using DNA methylation profiling, underscoring the diagnostic value of molecular techniques when histology alone is ambiguous. The patient was treated with BRAF and MEK inhibitor combination therapy, representing an actionable targeted approach made possible by identification of the driver mutation. Liquid biopsy was employed to non-invasively monitor treatment response and track circulating tumor DNA, demonstrating its potential utility in managing aggressive or metastatic circumscribed gliomas. This report highlights the importance of integrative molecular diagnostics and targeted therapy in rare, high-risk variants of PXA that progress beyond the central nervous system.

Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology

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2026-09-07 • AI

Deep Learning Pipeline for Automatic Segmentation, Classification, and Molecular Subtyping of Three Pediatric Posterior Fossa Tumors Using T2-Weighted MRI.

Jin Y, et al

A fully automated deep learning pipeline using standard T2-weighted MRI has been developed and validated to segment, classify, and molecularly subtype three pediatric posterior fossa tumors — medulloblastoma, ependymoma, and pilocytic astrocytoma — achieving tumor segmentation Dice scores of 0.94–0.96 and tumor-type classification accuracy of 0.824–0.918 (Cohen's kappa 0.722–0.873) across internal and external test sets in a multicenter cohort of 1,305 patients. Separate models for molecular subtyping reached an accuracy of 0.794 (Cohen's kappa 0.605) for medulloblastoma and 0.789 (Cohen's kappa 0.538) for ependymoma. This noninvasive, fully automated approach could enable preoperative differentiation of tumor types and molecular subtypes in children without the need for surgical sampling, directly informing treatment planning and prognosis.

Journal of magnetic resonance imaging : JMRI

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