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Although the genetic landscape of meningiomas has been extensively studied, the molecular alterations underlying ONSM remain poorly understood. We retrospectively analyzed consecutive patients surgically treated for ONSM between 2000 and 2025 at our institution, with available histological specimens. Intracranial meningiomas secondarily extending into the optic canal were excluded. Fresh\u2010frozen tumor samples were subjected to whole\u2010exome sequencing, and transcriptomic analyses were conducted and compared with those of intracranial meningiomas from four public datasets. Six cases were included, five of whom were female, with a median age of 63.5\u2009years. While most cases remained stable after surgery, one patient experienced multiple recurrences and ultimately succumbed. Primary tumors were characterized by the absence of\n                    <jats:italic>NF2<\/jats:italic>\n                    alterations, occasional\n                    <jats:italic>POLR2A<\/jats:italic>\n                    mutations, and few copy number variations (CNVs). Transcriptomic profiling in primary tumors revealed a neurotrophic microenvironment reflective of the close association with the optic nerve. The recurrent case exhibited high\u2010risk CNVs at diagnosis and developed into an aggressive disease as additional CNV burdens accumulated, including the homozygous deletion of\n                    <jats:italic>CDKN2A\/B<\/jats:italic>\n                    . Its expression profile was in line with that of hypermitotic, proliferative intracranial meningiomas. ONSM represents a predominantly\n                    <jats:italic>NF2<\/jats:italic>\n                    \u2010intact meningioma subtype defined by neural niche\u2010associated transcriptional signatures. Although typically indolent, ONSM can, in rare instances, evolve into an aggressive disease through further accumulation of CNVs.\n                  <\/jats:p>","DOI":"10.1111\/bpa.70078","type":"journal-article","created":{"date-parts":[[2026,2,2]],"date-time":"2026-02-02T06:28:27Z","timestamp":1770013707000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Optic nerve sheath meningioma exhibits neural niche\u2010associated transcriptomic features and rare\n                    <scp>copy number variation<\/scp>\n                    \u2010linked evolution"],"prefix":"10.1111","volume":"36","author":[{"given":"Daisuke","family":"Sato","sequence":"first","affiliation":[{"name":"Department of Neurosurgery The University of Tokyo  Tokyo 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