{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,7]],"date-time":"2026-08-07T16:34:40Z","timestamp":1786120480241,"version":"build-2736575974"},"reference-count":20,"publisher":"Wiley","license":[{"start":{"date-parts":[[2026,8,6]],"date-time":"2026-08-06T00:00:00Z","timestamp":1785974400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2026,8,6]],"date-time":"2026-08-06T00:00:00Z","timestamp":1785974400000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"funder":[{"DOI":"10.13039\/501100007052","name":"Universit\u00e0 degli Studi di Verona","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100007052","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100008088","name":"Fondazione I.R.C.C.S. Istituto Neurologico Carlo Besta","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100008088","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Brain Pathology"],"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    Primary meningeal melanocytic tumors (PMMTs) are rare neoplasms currently stratified by the World Health Organization (WHO) into three grades of malignancy based exclusively on histopathological criteria, while the prognostic significance of molecular alterations remains poorly defined. This study aimed to investigate the prognostic relevance of histopathological and molecular features in PMMTs and to assess the diagnostic utility of immunohistochemical and molecular markers for distinguishing PMMTs from malignant melanotic nerve sheath tumors (MMNSTs). Thirty\u2010six primary central nervous system (CNS) melanocytic tumors, including 24 PMMTs (10 grade 1, 12 grade 2, and 2 grade 3) and 12\u2009MMNSTs, were analyzed through integrated histopathological, immunohistochemical, genetic, and epigenetic characterization. Among grade 2 PMMTs, descriptively defined CNS\u2010invasive otherwise benign melanocytomas defined a clinically favorable subgroup, whereas\n                    <jats:italic>SF3B1<\/jats:italic>\n                    mutations and chromosome 8q gains were associated with higher recurrence rates and shorter recurrence\u2010free survival. Losses of chromosome 17 or 21q were observed exclusively in MMNSTs, supporting their potential diagnostic utility in distinguishing MMNSTs from PMMTs. These findings demonstrate biological heterogeneity among intermediate\u2010grade PMMTs and identify molecular markers associated with adverse clinical outcomes. Integrated histopathological and molecular characterization may enhance prognostic stratification and diagnostic accuracy in primary CNS melanocytic tumors.\n                  <\/jats:p>","DOI":"10.1111\/bpa.70133","type":"journal-article","created":{"date-parts":[[2026,8,6]],"date-time":"2026-08-06T23:11:46Z","timestamp":1786057906000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Molecular heterogeneity and prognostic biomarkers in primary meningeal melanocytic tumors"],"prefix":"10.1111","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9690-3500","authenticated-orcid":false,"given":"Rosina","family":"Paterra","sequence":"first","affiliation":[{"name":"Unit of Anatomic Pathology \u2010 Neuropathology Fondazione IRCCS Istituto Neurologico Carlo Besta  Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3567-9263","authenticated-orcid":false,"given":"Monica","family":"Patan\u00e8","sequence":"additional","affiliation":[{"name":"Unit of Anatomic Pathology \u2010 Neuropathology Fondazione IRCCS Istituto Neurologico Carlo Besta  Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0009-4803-1002","authenticated-orcid":false,"given":"Anna","family":"Stroppa","sequence":"additional","affiliation":[{"name":"Unit of Anatomic Pathology \u2010 Neuropathology Fondazione IRCCS Istituto Neurologico Carlo Besta  Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4508-3359","authenticated-orcid":false,"given":"Gianluca","family":"Marucci","sequence":"additional","affiliation":[{"name":"Unit of Anatomic Pathology \u2010 Neuropathology Fondazione IRCCS Istituto Neurologico Carlo Besta  Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1800-0896","authenticated-orcid":false,"given":"Serena","family":"Ammendola","sequence":"additional","affiliation":[{"name":"Unit of Pathology Azienda Ospedaliera Universitaria Integrata Verona  Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5035-2448","authenticated-orcid":false,"given":"Sofia","family":"Asioli","sequence":"additional","affiliation":[{"name":"Department of Biomedical and Neuromotor Sciences (DIBINEM) \u2010 Alma Mater Studiorum University of Bologna  Bologna Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7086-1920","authenticated-orcid":false,"given":"Valeria","family":"Barresi","sequence":"additional","affiliation":[{"name":"Unit of Anatomic Pathology \u2010 Neuropathology Fondazione IRCCS Istituto Neurologico Carlo Besta  Milan Italy"},{"name":"Dipartimento di Diagnostica e Sanit\u00e0 Pubblica Universit\u00e0 di Verona  Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2026,8,6]]},"reference":[{"issue":"4","key":"e_1_2_12_2_1","doi-asserted-by":"crossref","first-page":"iv1","DOI":"10.1093\/neuonc\/noaf194","article-title":"CBTRUS statistical report: primary brain and other central nervous system tumors diagnosed in the United States in 2018\u20102022","volume":"27","author":"Price M","year":"2025","journal-title":"Neuro Oncol"},{"key":"e_1_2_12_3_1","volume-title":"WHO classification of tumours central nervous system tumours","author":"Gessi M","year":"2021"},{"issue":"7","key":"e_1_2_12_4_1","doi-asserted-by":"crossref","first-page":"745","DOI":"10.1097\/00000478-199907000-00001","article-title":"Primary melanocytic neoplasms of the central nervous systems","volume":"23","author":"Brat DJ","year":"1999","journal-title":"Am J Surg Pathol"},{"issue":"14","key":"e_1_2_12_5_1","doi-asserted-by":"crossref","DOI":"10.3390\/cancers16142508","article-title":"Primary meningeal melanocytic tumors of the central nervous system: a review from the ultra\u2010rare brain tumors task force of the European network for rare cancers (EURACAN)","volume":"16","author":"Pellerino A","year":"2024","journal-title":"Cancers (Basel)"},{"issue":"2","key":"e_1_2_12_6_1","doi-asserted-by":"crossref","first-page":"202","DOI":"10.1111\/bpa.12228","article-title":"Melanotic tumors of the nervous system are characterized by distinct mutational, chromosomal and epigenomic profiles","volume":"25","author":"Koelsche C","year":"2015","journal-title":"Brain Pathol"},{"issue":"2","key":"e_1_2_12_7_1","doi-asserted-by":"crossref","first-page":"439","DOI":"10.1007\/s12253-014-9841-3","article-title":"Mutations in g protein encoding genes and chromosomal alterations in primary leptomeningeal melanocytic 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