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Optic pathway glioma is a slow-growing childhood brain tumour of the nerves that carry sight, often in children with neurofibromatosis type 1. It rarely kills but can take away vision, so treatment aims to preserve sight: watching if stable, chemotherapy such as carboplatin and vincristine if vision is threatened, or the MEK-blocking tablet selumetinib, with radiotherapy avoided in young children.
Optic pathway gliomas are pilocytic or other low-grade astrocytomas of the optic nerves, chiasm and hypothalamus, sporadic or associated with neurofibromatosis type 1, and treated within the paediatric low-grade glioma pathway (NCI PDQ). Because visual acuity rather than tumour size is what matters, the Response Evaluation in Neurofibromatosis and Schwannomatosis committee recommended quantitative visual acuity (Teller cards, then HOTV) as the main functional outcome for trials (Neurology 2013). In a single-institution series of 43 children treated with chemotherapy or radiotherapy, about 14 percent improved their vision during therapy and site and age strongly predicted long-term visual outcome (Cancer 2015). The MEK inhibitor selumetinib produced responses in recurrent or progressive NF1-associated and BRAF-aberrant paediatric low-grade glioma in the Pediatric Brain Tumor Consortium phase 2 trial (Fangusaro 2019), and MEK and RAF inhibitors are now first-line options on the parent page.
How it differs from its parent: the goal is sight, not tumour shrinkage; NF1-associated tumours are usually not biopsied and follow a gentler course; radiotherapy is avoided because of vascular and second-tumour risks in NF1 and cognitive harm in young children.
How common: no registry figure in the sources read.
Treatment: observation with ophthalmology follow-up when stable; carboplatin and vincristine chemotherapy or a MEK inhibitor (selumetinib) or RAF inhibitor (tovorafenib, dabrafenib with trametinib for BRAF V600E) when vision is threatened or the tumour progresses; surgery only for a blind, painful proptotic eye or hydrocephalus; radiotherapy deferred as long as possible.
A common form of paediatric low-grade glioma and the commonest brain tumour of neurofibromatosis type 1, in which it arises in about 15 to 20 percent of children by the estimates the trial literature uses; no registry figure is in the sources read.
Uveal melanoma arises in the pigmented choroid and ciliary body, retinoblastoma in the retina of infants; the eye has no lymphatics, so spread is through the blood (uveal melanoma almost always to the liver).
No lymphatic drainage: spread is haematogenous (uveal melanoma to the liver) or along the optic nerve (retinoblastoma).
Same organ: Conjunctival melanoma, Uveal melanoma, Retinoblastoma
Carboplatin and vincristine, or selumetinib (phase 2, Fangusaro 2019), tovorafenib, or dabrafenib with trametinib for BRAF V600E; radiotherapy deferred.
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Query for this cancer: (TITLE:"Optic pathway glioma" OR ABSTRACT:"Optic pathway glioma" OR TITLE:"Optic nerve glioma" OR ABSTRACT:"Optic nerve glioma" OR TITLE:"Optic glioma" OR ABSTRACT:"Optic glioma" OR TITLE:"NF1-associated optic pathway glioma" OR ABSTRACT:"NF1-associated optic pathway glioma" OR TITLE:"Hypothalamic-chiasmatic glioma" OR ABSTRACT:"Hypothalamic-chiasmatic glioma" OR TITLE:"NF1-associated optic pathway glioma paediatric low-grade glioma" OR ABSTRACT:"NF1-associated optic pathway glioma paediatric low-grade glioma") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Optic pathway glioma, not a curated reading list.
The targets of this cancer's medicines and the ones linked to it directly.
Cases by country, the UK and NHS pathway and other country lenses, and the expert centres with trials on record.
One section per setting: the options named, what each is for, the trials behind them, the recorded trade-offs and the questions to ask.
Fainting, near-fainting, or an irregular or racing heartbeat; several kinase inhibitors prolong the QT interval and the labels require ECG and electrolyte monitoring.
Bleeding that will not stop, black or bloody stools, or unexplained bruising when platelets are expected to be low.
Fatal if given intrathecally: label all syringes.
Dose by Calvert formula using GFR (see the calculators).
Reactions around the moment a drug is given: chills, fever or breathlessness from antibodies (infusion reactions), true allergy (hypersensitivity, rarely anaphylaxis), and leakage of a damaging drug into tissue around the vein (extravasation).
During chemotherapy for triple-negative breast cancer a temperature over 37.5 C or below 36 C, shivering, or feeling unwell even with a normal temperature means ringing the hospital's 24-hour line straight away; breathing very fast, confusion, mottled skin or no urine in a day means 999.
See all on the product pages:CarboplatinDabrafenibSelumetinibTrametinibVincristine·Printable cards in the navigator
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Everything in development, the open problems and what is being done about them, the roadmaps, and what changed on this record.
Every connected record, the notes, the JSON, Markdown and RDF twins, and where the record came from and when it was checked.