Optic nerve gliomas (ONGs) in children are uniformly low-grade gliomas that frequently cause vision loss. Optic nerve gliomas in adults are reported to be more clinically aggressive with frequent transformation into high-grade gliomas. We report a child with sporadic ONG and an aggressive clinical course. Tumor molecular characterization identified KIAA1549-BRAF fusion but also set domain-containing protein 2 (SETD2; OMIM, 612778) mutation, previously reported only, to our knowledge, in high-grade gliomas and non–central nervous system cancers.
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Campbell AA, Gartrell-Corrado RD, Mansukhani M, et al. SETD2 Mutation in an Aggressive Optic Nerve Glioma. JAMA Ophthalmol. 2020;138(1):102–104. doi:10.1001/jamaophthalmol.2019.4511
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