Optic Nerve Sheath Meningiomas: A Narrative Review of Clinical Features, Imaging Advances, and Current Management

Optic nerve sheath meningioma (ONSM) is a rare orbital tumor that can pose a significant threat to visual function due to its close relationship with the optic nerve and surrounding vascular structures. Early and accurate diagnosis is essential to preserve vision and guide individualized management. The objective of this narrative review is to provide a comprehensive overview of the current evidence on the clinical presentation, diagnostic evaluation, imaging characteristics, and management of ONSM, with a particular focus on recent advances in imaging techniques and their implications for clinical decision-making. This review synthesizes current knowledge on clinical presentation, diagnostic workup, and evolving treatment strategies for ONSM, with particular emphasis on advanced imaging modalities. Magnetic resonance imaging remains the cornerstone of diagnosis, yet functional imaging, especially somatostatin receptor-–based techniques such as 68GaDOTATATE PET/CT have shown potential as a useful adjunct in the diagnostic evaluation of ONSM, especially for assessing tumor characteristics, extent, and treatment planning in selected cases. We discuss the emerging role of DOTATATE imaging in differentiating ONSM from other optic nerve and orbital pathologies, as well as its potential application in monitoring therapeutic response. We also highlight contemporary management approaches, including fractionated radiotherapy and the selective role of surgical intervention, underscoring the importance of multidisciplinary decision-making in optimizing visual outcomes. Emerging molecular insights from meningioma research may inform future therapeutic strategies for ONSM; however, clinical evidence specific to ONSM remains limited.