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Optic pathway glioma – NF1 — RACP Paediatrics MCQ

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ModerateNeurologyOptic pathway glioma – NF1RACP Paediatrics

A 6-year-old with known NF1 presents with progressive proptosis and visual loss in the right eye. MRI shows a fusiform enlargement of the right optic nerve extending to the optic chiasm. What is the most likely tumour?

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Correct answer: C — Optic pathway glioma

Optic pathway glioma (pilocytic astrocytoma) is associated with NF1 (~15% of NF1 patients). They are typically low-grade and may be asymptomatic for years. Treatment is indicated for progressive visual loss or symptomatic growth. Chemotherapy (carboplatin/vincristine) is first-line. Radiation is avoided in NF1 due to secondary malignancy risk.

Reference: RCH Melbourne – 2023 – NF1 CPG; RACP Paediatric Curriculum – Oncology