Optic Nerve Sheath Meningiomas

Optic nerve sheath meningiomas (ONSMs) grow slowly and, if untreated, patients may have stable visual function for up to several years. Treatment of an ONSM may lead to vision loss (radiation retinopathy or optic neuropathy). Therefore, observation is recommended for a patient with ONSM and relatively preserved visual acuity, color vision, pupils, and visual fields. Follow-up every 4 to 6 months initially is recommended extending to annual examinations if visual function and tumor size remain stable for a few years. Neuroimaging can be repeated every 12 months. An undisputed decline in visual function or any intracranial extension warrants treatment of the ONSM. The treatment of choice for a tumor confined to the orbit is stereotactic fractionated radiation. Stereotactic fractionated radiation uses multiple small doses of radiation using tight margins. A reasonable alternative, three-dimensional conformal fractionated radiation uses computed tomography-guided planning but usually requires wider margins. Conventional radiation uses much wider margins and would not be recommended for treatment of ONSM. The radiation can be administered during 5 to 6 weeks in 28 daily fractions of 1.8 to 2 Gy/fraction to a total of 50.4 to 56 Gy. Many patients have improvement or stabilization of their visual function. Gamma knife radiosurgery does not have a role in ONSM because the required dose is toxic to the optic nerve. A tumor that extends intracranially may be treated with fractionated radiation if any vision remains. Surgical excision can be considered for significant intracranial extension but this often leads to complete vision loss in the ipsilateral eye. A blind, disfigured eye also may be treated with en bloc surgical resection of the meningioma.