A case of rhabdoid meningioma originating from the optic nerve

• DOI: 10.1111/neup.13028
• Publication Date: 2025-01-27T07:20:17Z
• Authors: Jing Liu; Ziling Yan; Fan Lin; Xia Liu

We report a rare case of rhabdoid meningioma (RM) originating from the optic nerve in a 57‐year‐old female. The tumor exhibited rhabdoid or epithelioid histology and harbored BAP1 inactivation mutations. Optic nerve meningioma typically originates from the outer meningeal cells of the optic nerve within the optic canal and is usually benign, with most cases classified as meningothelial or transitional meningiomas. This is the first reported case of RM involving the optic nerve, presenting with World Health Organization (WHO) central nervous system (CNS) grade 1 histological features but without CDKN2A/B homozygous deletions or telomerase reverse transcriptase promoter mutations, though harboring a BAP1 deletion. Despite being classified as a low‐grade tumor by current standards, the rapid recurrence and progression observed underscore the importance of reporting this case to enhance awareness among pathologists and reduce misdiagnoses.