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Optic gliomas may unpredictably stabilize or progress. Prognosis was known to be better in patients with neurofibromatosis 1 (NF1) than those with non-NF1. The confusion over management is caused, at least in part, by the uncertain disease course of the tumor.We report here a 13-year-old girl presented with a 1-month history of a gradual decline of vision in her left eye. She was diagnosed by clinical examination and magnetic resonance imaging as having a left optic-nerve glioma and was followed by serial magnetic resonance imaging over 3 years. She and her family did not show any stigmata of NF1. Approximately 3 years after the onset of her symptoms, visual acuity, color testing, and visual fields have markedly improved with tumor regression by magnetic resonance imaging.The possibility of spontaneous regression of an optic-nerve glioma should be considered even in the planning of treatment for a non-NF1 patient with this tumor.