2 yo M presenting with L weakness, peripheral vision deterioration and head tilt found to have a ~6x6 cm suprasellar mass by MRI brain c/w optic pathway glioma w/o signs of NF1. Biopsy preliminary findings: low-grade astrocytoma with piloid features.
Final diagnosis: CNS WHO Grade 1, angiocentric glioma.
Methylation Superfamily: low grade glial/glioneuronal tumor (0.988),
Methylation Class: Diffuse astrocytoma, MYB/MYBL1-altered, subtype B (0.655)
Unmethylated.
Glioseq: no genomic alterations.
Discuss Treatment recommendations:
Prefer tumor directed treatment over surgery given age and location of tumor.
Considering Carbo/VCR, but wondering is anyone would consider oral inhibition?
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Sheila McThenia
Assistant Professor, Pediatrics
Children's Healthcare of Atlanta
Atlanta GA
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