Pediatric Low-Grade Gliomas Management and Outcomes

Pediatric low-grade gliomas (pLGGs) represent the most common central nervous system tumours in children, encompassing WHO grade I and II neoplasms with generally indolent behaviour. The prototypical histology is pilocytic astrocytoma, frequently arising in the cerebellum, optic pathways or diencephalic region. Management is predicated on maximising safe surgical resection, which remains the cornerstone of treatment and offers the best chance of long-term disease control. In cases where complete excision is unachievable, conventional chemotherapy regimens—most often vincristine and carboplatin—are employed to arrest progression while deferring radiotherapy until later childhood to mitigate neurocognitive sequelae. Advances in molecular profiling have revealed ubiquitous activation of the MAPK/ERK signalling axis, paving the way for targeted agents such as MEK inhibitors and RAF inhibitors. Anti-angiogenic strategies and immunomodulatory approaches are under active investigation, with the dual aims of improving progression-free survival and preserving neurological, visual and endocrine function. Overall survival at five years exceeds 90 per cent in most series, yet progression-free survival remains around 50–60 per cent, and long-term morbidity from visual impairment, hypothalamic dysfunction and cognitive late effects underscores the need for multidisciplinary follow-up.

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Pediatric Low-Grade Gliomas Management and Outcomes publication trend

The graph below shows the total number of articles in pediatric low-grade gliomas management and outcomes across all publications each year (not limited to Nature Index journals).

Technical terms

Low-grade glioma: A World Health Organization grade I or II primary brain tumour with relatively slow growth and favourable prognosis.

Pilocytic astrocytoma: A circumscribed, slow-growing astrocytic tumour most common in children, often exhibiting cystic features and Rosenthal fibres.

MAPK/ERK pathway: A signalling cascade (mitogen-activated protein kinase/extracellular signal-regulated kinase) frequently hyperactivated in pLGGs via BRAF mutations or fusions.

MEK inhibitor: A targeted small molecule that blocks MEK1/2 kinases in the MAPK pathway to halt tumour cell proliferation.

BRAF fusion: A genetic rearrangement fusing BRAF to partner genes (commonly KIAA1549) that constitutively activates downstream signalling.

Gross total resection: Surgical removal of all visible tumour, associated with the highest rates of durable control in pLGG.

Progression-free survival: The interval during which a patient remains alive without radiographic or clinical evidence of tumour progression.

Optic pathway glioma: A glioma involving the optic nerves, chiasm or tracts, often associated with visual loss and endocrine dysfunction in children.

References

1. The Present and Future of Optic Pathway Glioma Therapy. Cells (2023).
2. Pediatric Low-Grade Gliomas. Cancers (2020).
3. LOGGIC/FIREFLY-2: a phase 3, randomized trial of tovorafenib vs. chemotherapy in pediatric and young adult patients with newly diagnosed low-grade glioma harboring an activating RAF alteration. BMC Cancer (2024).
4. Neurosurgical experience of managing optic pathway gliomas. Child's Nervous System (2021).