{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,15]],"date-time":"2026-09-15T15:50:20Z","timestamp":1789487420492,"version":"build-2803163510"},"reference-count":28,"publisher":"Wiley","issue":"10","license":[{"start":{"date-parts":[[2026,7,23]],"date-time":"2026-07-23T00:00:00Z","timestamp":1784764800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"},{"start":{"date-parts":[[2026,7,23]],"date-time":"2026-07-23T00:00:00Z","timestamp":1784764800000},"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":["Molecular Carcinogenesis"],"published-print":{"date-parts":[[2026,10]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:label\/>\n                    <jats:p>BRCA1\u2010associated protein 1 (BAP1)\u2010deficient meningiomas represent a clinically and molecularly distinct subgroup with variable histology and aggressive behavior that may not be fully captured by current grading criteria. The clinical spectrum of BAP1 alterations in meningiomas, including both somatic and germline contexts, remains incompletely characterized. We report three cases of BAP1\u2010deficient meningiomas with divergent clinical trajectories. Case 1 was a 44\u2010year\u2010old woman with a somatic BAP1 nonsense mutation (Q392*) presenting as recurrent spinal meningiomatosis. Despite WHO grade 1 histology with rhabdoid features and no high\u2010grade criteria, she experienced rapid recurrence and required multiple resections, radiation, and systemic therapy, illustrating that truncating somatic mutations may drive aggressive behavior independent of histological grade. Case 2 was a 22\u2010year\u2010old man harboring a germline BAP1 truncating mutation (R385*) with a family history of meningioma, who presented with a WHO grade 3 papillary meningioma and remained progression\u2010free 24 months after resection and adjuvant radiotherapy. His family pedigree represents a rare phenotype within the BAP1 tumor predisposition syndrome. Case 3 was a 57\u2010year\u2010old man with a low\u2010variant\u2010allele\u2010frequency BAP1 missense mutation (Y173C) and WHO grade 2 meningioma who demonstrated a favorable course at 26 months following gross total resection without adjuvant therapy. These cases illustrate the phenotypic heterogeneity of BAP1\u2010deficient meningiomas and underscore that truncating mutations, particularly somatic ones, may confer aggressive behavior regardless of histological grade. Variant allele frequency and mutation type should inform risk stratification alongside traditional pathological features. Consideration of BAP1 as a defining molecular feature in future WHO classification iterations is likely warranted.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/mc.70146","type":"journal-article","created":{"date-parts":[[2026,7,23]],"date-time":"2026-07-23T12:19:48Z","timestamp":1784809188000},"page":"1343-1350","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Clinical and Molecular Features of BAP1\u2010Mutated Meningiomas: Case Series"],"prefix":"10.1002","volume":"65","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6065-4781","authenticated-orcid":false,"given":"Ivan","family":"Pradilla","sequence":"first","affiliation":[{"name":"Department of Neuro\u2010Oncology The University of Texas MD Anderson Cancer Center Houston Texas USA"},{"name":"Instituto M\u00e9deri del Cerebro, Cabeza y Cuello, Hospital Universitario Mayor, M\u00e9deri Universidad del Rosario Bogot\u00e1 Colombia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rasha","family":"Alfattal","sequence":"additional","affiliation":[{"name":"Molecular Genetic Pathology, Department of Pathology The University of Texas MD Anderson Cancer Center Houston Texas USA"},{"name":"Neuropathology, Department of Pathology Houston Methodist Hospital Houston Texas USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5286-0649","authenticated-orcid":false,"given":"Susan L.","family":"McGovern","sequence":"additional","affiliation":[{"name":"Department of Radiation Oncology, The University of Texas MD Anderson Cancer Center University of Texas Houston Texas USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shaan M.","family":"Raza","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, The University of Texas MD Anderson Cancer Center University of Texas Houston Texas USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Franco","family":"DeMonte","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, The University of Texas MD Anderson Cancer Center University of Texas Houston Texas USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6854-6830","authenticated-orcid":false,"given":"Leomar Y.","family":"Ballester","sequence":"additional","affiliation":[{"name":"Molecular Genetic Pathology, Department of Pathology The University of Texas MD Anderson Cancer Center Houston Texas USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chirag B.","family":"Patel","sequence":"additional","affiliation":[{"name":"Department of Neuro\u2010Oncology The University of Texas MD Anderson Cancer Center Houston Texas USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carlos","family":"Kamiya\u2010Matsuoka","sequence":"additional","affiliation":[{"name":"Department of Neuro\u2010Oncology The University of Texas MD Anderson Cancer Center Houston Texas USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2026,7,23]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1093\/neuonc\/nox094"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-016-1545-1"},{"key":"e_1_2_10_4_1","unstructured":"WHO Classification of Tumours Editorial Board ed. WHO Classification of Tumors: Central Nervous System Tumours vol6.5th ed 2021."},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.3390\/biology13050350"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1093\/neuonc\/noaf105"},{"key":"e_1_2_10_7_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41418-020-00709-4"},{"key":"e_1_2_10_8_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1309085110"},{"key":"e_1_2_10_9_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.1194472"},{"key":"e_1_2_10_10_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng.910"},{"key":"e_1_2_10_11_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng.912"},{"key":"e_1_2_10_12_1","doi-asserted-by":"publisher","DOI":"10.1136\/jmedgenet-2011-100156"},{"key":"e_1_2_10_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ajhg.2013.04.012"},{"key":"e_1_2_10_14_1","doi-asserted-by":"publisher","DOI":"10.1093\/neuonc\/nou148"},{"key":"e_1_2_10_15_1","doi-asserted-by":"publisher","DOI":"10.4103\/1793-5482.145185"},{"key":"e_1_2_10_16_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.clineuro.2021.106766"},{"key":"e_1_2_10_17_1","doi-asserted-by":"publisher","DOI":"10.1093\/neuonc\/noac137"},{"key":"e_1_2_10_18_1","doi-asserted-by":"publisher","DOI":"10.1093\/jnci\/djy171"},{"key":"e_1_2_10_19_1","doi-asserted-by":"publisher","DOI":"10.3389\/fonc.2024.1338022"},{"key":"e_1_2_10_20_1","doi-asserted-by":"publisher","DOI":"10.1093\/neuonc\/now235"},{"key":"e_1_2_10_21_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.canlet.2016.05.011"},{"key":"e_1_2_10_22_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0035295"},{"key":"e_1_2_10_23_1","doi-asserted-by":"publisher","DOI":"10.1186\/s40364-015-0040-5"},{"key":"e_1_2_10_24_1","doi-asserted-by":"publisher","DOI":"10.1136\/jclinpath-2021-207952"},{"key":"e_1_2_10_25_1","doi-asserted-by":"publisher","DOI":"10.3390\/cancers11081114"},{"key":"e_1_2_10_26_1","doi-asserted-by":"publisher","DOI":"10.1001\/jamadermatol.2017.2330"},{"key":"e_1_2_10_27_1","doi-asserted-by":"publisher","DOI":"10.3389\/fonc.2021.721712"},{"key":"e_1_2_10_28_1","doi-asserted-by":"publisher","DOI":"10.3340\/jkns.2016.59.4.357"},{"key":"e_1_2_10_29_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41525-020-00157-6"}],"container-title":["Molecular Carcinogenesis"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/mc.70146","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/mc.70146","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/mc.70146","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,9,15]],"date-time":"2026-09-15T14:29:23Z","timestamp":1789482563000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/mc.70146"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,7,23]]},"references-count":28,"journal-issue":{"issue":"10","published-print":{"date-parts":[[2026,10]]}},"alternative-id":["10.1002\/mc.70146"],"URL":"https:\/\/doi.org\/10.1002\/mc.70146","archive":["Portico"],"relation":{},"ISSN":["0899-1987","1098-2744"],"issn-type":[{"value":"0899-1987","type":"print"},{"value":"1098-2744","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,7,23]]},"assertion":[{"value":"2026-02-13","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-06-23","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-07-23","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}