{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,24]],"date-time":"2026-09-24T13:04:18Z","timestamp":1790255058088,"version":"4.1.0"},"reference-count":51,"publisher":"Wiley","issue":"9","license":[{"start":{"date-parts":[[2026,9,21]],"date-time":"2026-09-21T00:00:00Z","timestamp":1789948800000},"content-version":"vor","delay-in-days":20,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2026,9,1]],"date-time":"2026-09-01T00:00:00Z","timestamp":1788220800000},"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":["Genes Chromosomes &amp;amp; Cancer"],"published-print":{"date-parts":[[2026,9]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>\n                    Malignant peripheral nerve sheath tumor (MPNST) carries the highest excess mortality of the cancers associated with neurofibromatosis Type 1, and usually arises from a neurofibroma. We asked whether the transcriptomic differences that distinguish MPNST from neurofibroma reproduce across independent cohorts, and whether a compact score derived from them transports between platforms. We meta\u2010analyzed five public discovery cohorts of primary nerve\u2010sheath tissue (113 MPNST, 79 neurofibromas; 3 platforms) and held out a sixth, independently generated cohort. Neurofibroma and MPNST tissue differed at 1489 genes in two opposing themes: up\u2010regulated mitotic machinery (\n                    <jats:italic>TOP2A<\/jats:italic>\n                    ,\n                    <jats:italic>EZH2<\/jats:italic>\n                    ,\n                    <jats:italic>AURKA<\/jats:italic>\n                    ) and loss of Schwann\u2010cell identity (the transcription factors\n                    <jats:italic>SOX10<\/jats:italic>\n                    and\n                    <jats:italic>EGR2<\/jats:italic>\n                    , and the myelin genes\n                    <jats:italic>PMP2<\/jats:italic>\n                    and\n                    <jats:italic>MPZ<\/jats:italic>\n                    ). These differences were reproducible. Among the significant genes assayed in all five cohorts, almost all changed in the same direction in everyone, and 93.3% of those detectable in the held\u2010out cohort agreed in direction. Leave\u2010one\u2010cohort\u2010out re\u2010derivation of the pipeline gave a mean AUC of 0.90 across five folds (per\u2010fold 0.84 to 0.97).\n                    <jats:italic>CD276<\/jats:italic>\n                    (B7\u2010H3) was selectively up\u2010regulated, whereas\n                    <jats:italic>CD274<\/jats:italic>\n                    and\n                    <jats:italic>PDCD1LG2<\/jats:italic>\n                    were not. The myelin marker\n                    <jats:italic>PMP2<\/jats:italic>\n                    , a readout chosen retrospectively after the held\u2010out results were known, separated MPNST from neurofibroma in that cohort but left atypical and conventional neurofibromas unseparated (AUC 0.67,\n                    <jats:italic>p<\/jats:italic>\n                    \u2009=\u20090.10). A program score derived without the discovery cohort carrying survival data was associated with shorter survival there, an exploratory single\u2010cohort association. The composition of a compact score varied with platform and preprocessing, so no fixed single\u2010sample classifier is warranted.\n                  <\/jats:p>","DOI":"10.1002\/gcc.70175","type":"journal-article","created":{"date-parts":[[2026,9,21]],"date-time":"2026-09-21T12:55:04Z","timestamp":1789995304000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["A Reproducible Transcriptomic Program of Neurofibroma\u2010to\u2010Malignant Peripheral Nerve Sheath Tumor Transformation"],"prefix":"10.1002","volume":"65","author":[{"ORCID":"https:\/\/orcid.org\/0009-0007-5506-8298","authenticated-orcid":false,"given":"Quanyu","family":"Jin","sequence":"first","affiliation":[{"name":"Department of Neurosurgery China\u2010Japan Friendship Hospital (Institute of Clinical Medical Sciences), Chinese Academy of Medical Sciences &amp; Peking Union Medical College  Beijing China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0004-0598-0762","authenticated-orcid":false,"given":"Zhenxuan","family":"Gao","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery China\u2010Japan Friendship Hospital (Institute of Clinical Medical Sciences), Chinese Academy of Medical Sciences &amp; Peking Union Medical College  Beijing China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2050-1761","authenticated-orcid":false,"given":"Ze","family":"Zhang","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery Peking University China\u2010Japan Friendship School of Clinical Medicine  Beijing China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chenchen","family":"Kong","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery China\u2010Japan Friendship Hospital (Institute of Clinical Medical Sciences), Chinese Academy of Medical Sciences &amp; Peking Union Medical College  Beijing China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0005-0162-3554","authenticated-orcid":false,"given":"Jiaxin","family":"Liu","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery China\u2010Japan Friendship Hospital  Beijing China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aerman","family":"Abudurezhake","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery Peking University China\u2010Japan Friendship School of Clinical Medicine  Beijing China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lei","family":"Kou","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery Peking University China\u2010Japan Friendship School of Clinical Medicine  Beijing 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