{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,15]],"date-time":"2026-09-15T08:03:32Z","timestamp":1789459412136,"version":"build-2803163510"},"reference-count":19,"publisher":"Wiley","license":[{"start":{"date-parts":[[2026,9,15]],"date-time":"2026-09-15T00:00:00Z","timestamp":1789430400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"},{"start":{"date-parts":[[2026,9,15]],"date-time":"2026-09-15T00:00:00Z","timestamp":1789430400000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Brain Pathology"],"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    Circumscribed meningeal melanocytic neoplasms (CMMNs) represent a spectrum of rare central nervous system (CNS) tumors. Knowledge of their clinical behavior and molecular correlates remains limited. In this single\u2010institution retrospective study, we analyzed 31 patients (14 male, 17 female; median age, 59\u2009years) diagnosed with CMMN between 2004 and 2025. In this patient cohort, based on the current World Health Organization Central Nervous System Tumor Classification (WHO CNS5) criteria, the majority of cases (18, 58.1%) fell into the intermediate\u2010grade melanocytic tumor (IMT) category based on histology, with either increased mitotic activity, CNS invasion, or both; 7 (22.6%) were melanocytomas, 5 (16.1%) melanomas, and 1 (3.2%) indeterminate case because of limited tissue. By next\u2010generation sequencing, mutations were identified in\n                    <jats:italic>GNAQ\/GNA11<\/jats:italic>\n                    in 21\/24 cases,\n                    <jats:italic>BAP1<\/jats:italic>\n                    in 5\/23 (2 IMT, 3 melanoma),\n                    <jats:italic>EIF1AX<\/jats:italic>\n                    in 9\/21 (1 melanocytoma, 8 IMT), and\n                    <jats:italic>SF3B1<\/jats:italic>\n                    in 3\/23 (2 IMT, 1 melanoma). Primary sites were spinal (20), posterior fossa (6), and supratentorial (5). During follow\u2010up, progression was observed in 5\/6 melanocytomas, 9\/13 IMTs, and 4\/5 melanomas, suggesting a high frequency of progression across all groups. Two\u2010year progression\u2010free survival was 83.3% (95% confidence interval [CI], 53.5%\u2013100.0%) for melanocytoma, 55.9% (95% CI, 26.7%\u201385.2%) for IMT, and 25.0% (95% CI, 0.0%\u201367.4%) for melanoma. Five\u2010year overall survival (OS) was 80.0% (95% CI, 44.9%\u2013100.0%) for melanocytoma, 65.3% (95% CI, 37.0%\u201393.6%) for IMT, and 0.0% for melanoma. OS was significantly worse in melanoma compared with melanocytoma (\n                    <jats:italic>p<\/jats:italic>\n                    \u2009=\u20090.002).\n                    <jats:italic>BAP1<\/jats:italic>\n                    mutation correlated with worse OS (hazard ratio 8.73,\n                    <jats:italic>p<\/jats:italic>\n                    \u2009=\u20090.006). Upfront maximal safe resection appeared to be associated with improved outcomes in selected patients, whereas radiotherapy and systemic therapy did not demonstrate clear additional benefit in this cohort. In summary, our study shows that all CMMNs, irrespective of grade, have a high propensity to recur and melanoma histology and\n                    <jats:italic>BAP1<\/jats:italic>\n                    mutation are associated with significantly worse OS.\n                  <\/jats:p>","DOI":"10.1111\/bpa.70147","type":"journal-article","created":{"date-parts":[[2026,9,15]],"date-time":"2026-09-15T07:13:52Z","timestamp":1789456432000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Circumscribed Meningeal Melanocytic Neoplasms: CNS WHO Grade, Molecular Profile, and Clinical Outcomes"],"prefix":"10.1111","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1778-8880","authenticated-orcid":false,"given":"Yi","family":"Zhu","sequence":"first","affiliation":[{"name":"Department of Laboratory Medicine and Pathology Mayo Clinic  Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3360-6733","authenticated-orcid":false,"given":"Nishika","family":"Karbhari","sequence":"additional","affiliation":[{"name":"Department of Neurology Mayo Clinic  Rochester Minnesota USA"},{"name":"Department of Oncology Mayo Clinic  Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Timothy J.","family":"Kaufmann","sequence":"additional","affiliation":[{"name":"Department of Radiology Mayo Clinic  Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6198-7441","authenticated-orcid":false,"given":"Aditya","family":"Raghunathan","sequence":"additional","affiliation":[{"name":"Department of Laboratory Medicine and Pathology Mayo Clinic  Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2768-2913","authenticated-orcid":false,"given":"Cristiane M.","family":"Ida","sequence":"additional","affiliation":[{"name":"Department of Laboratory Medicine and Pathology Mayo Clinic  Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0434-4965","authenticated-orcid":false,"given":"Cinthya J.","family":"Zepeda\u2010Mendoza","sequence":"additional","affiliation":[{"name":"Department of Laboratory Medicine and Pathology Mayo Clinic  Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8590-2681","authenticated-orcid":false,"given":"Ian J.","family":"Dryden","sequence":"additional","affiliation":[{"name":"Department of Laboratory Medicine and Pathology Mayo Clinic  Jacksonville Florida USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shahriar","family":"Salamat","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine University of Wisconsin\u2010Madison  Madison Wisconsin USA"},{"name":"Department of Neurological Surgery University of Wisconsin\u2010Madison  Madison Wisconsin USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chathu","family":"Atherton","sequence":"additional","affiliation":[{"name":"Department of Oncology Mayo Clinic  Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sarah M.","family":"Jenkins","sequence":"additional","affiliation":[{"name":"Division of Clinical Trials and Biostatistics, Department of Quantitative Health Sciences Mayo Clinic  Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9238-4325","authenticated-orcid":false,"given":"Svetomir N.","family":"Markovic","sequence":"additional","affiliation":[{"name":"Department of Oncology Mayo Clinic  Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3919-7408","authenticated-orcid":false,"given":"Ugur T.","family":"Sener","sequence":"additional","affiliation":[{"name":"Department of Neurology Mayo Clinic  Rochester Minnesota USA"},{"name":"Department of Oncology Mayo Clinic  Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2757-6782","authenticated-orcid":false,"given":"Caterina","family":"Giannini","sequence":"additional","affiliation":[{"name":"Department of Laboratory Medicine and Pathology Mayo Clinic  Rochester Minnesota 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