{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,18]],"date-time":"2026-09-18T23:51:17Z","timestamp":1789775477488,"version":"4.0.1"},"reference-count":42,"publisher":"Wiley","issue":"9","license":[{"start":{"date-parts":[[2025,9,12]],"date-time":"2025-09-12T00:00:00Z","timestamp":1757635200000},"content-version":"vor","delay-in-days":11,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Genes Chromosomes &amp;amp; Cancer"],"published-print":{"date-parts":[[2025,9]]},"abstract":"<jats:title>ABSTRACT<\/jats:title><jats:p>Conventional and dedifferentiated chondrosarcoma encompass a group of malignant neoplasms that produce cartilaginous matrix and arise within or on the surface of bone. Conventional chondrosarcomas are graded on a three\u2010tiered scale, whereas dedifferentiated chondrosarcoma is typically not graded but is considered a high\u2010grade sarcoma and represents the most aggressive subtype with a poor prognosis. <jats:italic>IDH1<\/jats:italic> (isocitrate dehydrogenase\u20101) and <jats:italic>I<\/jats:italic><jats:italic>DH2<\/jats:italic> (isocitrate dehydrogenase\u20102) are the most commonly mutated genes in conventional and dedifferentiated chondrosarcoma, followed in frequency by <jats:italic>COL2A1<\/jats:italic> and <jats:italic>TP53<\/jats:italic>. <jats:italic>IDH1\/2<\/jats:italic> driver mutations are also commonly found in enchondroma, considered a benign precursor lesion of chondrosarcoma, and other malignancies such as gliomas, cholangiocarcinoma, and acute myeloid leukemia. In acute myeloid leukemia, the presence of concurrent <jats:italic>BCOR<\/jats:italic> (BCL\u20106 corepressor) loss\u2010of\u2010function mutations has been linked to disease relapse and resistance to treatment with IDH inhibitors. After identifying an index case of conventional chondrosarcoma with unusually aggressive clinical evolution, we investigated the clinicopathological features of 12 cases of <jats:italic>BCOR<\/jats:italic>\u2010mutated conventional and dedifferentiated chondrosarcomas against a control group of 15 <jats:italic>BCOR<\/jats:italic>\u2010wildtype (WT) cases to determine whether <jats:italic>BCOR<\/jats:italic>\u2010mutated tumors had patterns of biological progression different from tumors with intact <jats:italic>BCOR<\/jats:italic>. All identified <jats:italic>BCOR<\/jats:italic> alterations led to loss\u2010of\u2010function by either missense or nonsense mutations. The prevalence of <jats:italic>BCOR<\/jats:italic> mutations occurred in 5% of conventional and dedifferentiated chondrosarcoma, and these were associated with larger tumor size (<jats:italic>p<\/jats:italic>\u2009=\u20090.024), metastasis at the time of diagnosis (<jats:italic>p<\/jats:italic>\u2009\u2264\u20090.001) and higher T category (3\u20134 vs. 1\u20132) (<jats:italic>p<\/jats:italic>\u2009=\u20090.009). Although larger studies are necessary to clarify the full impact of <jats:italic>BCOR<\/jats:italic> mutations on patients with conventional and dedifferentiated chondrosarcoma, our data indicate that <jats:italic>BCOR<\/jats:italic> genetic aberrations are associated with adverse clinical features.<\/jats:p>","DOI":"10.1002\/gcc.70068","type":"journal-article","created":{"date-parts":[[2025,9,13]],"date-time":"2025-09-13T06:55:38Z","timestamp":1757746538000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["<scp>BCOR<\/scp>\u2010Mutated Conventional and Dedifferentiated Chondrosarcoma: A Clinicopathologic Study"],"prefix":"10.1002","volume":"64","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8557-7014","authenticated-orcid":false,"given":"Diego M.","family":"Montoya\u2010Cerrillo","sequence":"first","affiliation":[{"name":"Department of Pathology and Laboratory Medicine University of Miami Miller School of Medicine  Miami Florida USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9358-9843","authenticated-orcid":false,"given":"Mark G.","family":"Evans","sequence":"additional","affiliation":[{"name":"Caris Life Sciences  Irving Texas USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrew","family":"Elliott","sequence":"additional","affiliation":[{"name":"Caris Life Sciences  Irving Texas USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Renzo Calderon","family":"Anyosa","sequence":"additional","affiliation":[{"name":"Institute of Health Policy, Management and Evaluation, Dalla Lana School of Public Health University of Toronto  Toronto Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jaylou Velez","family":"Torres","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine University of Miami Miller School of Medicine  Miami Florida USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elizabeth A.","family":"Montgomery","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine University of Miami Miller School of Medicine  Miami Florida USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francis J.","family":"Hornicek","sequence":"additional","affiliation":[{"name":"Division of Orthopedic Oncology University of Miami Miller School of Medicine  Miami Florida USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"H. Thomas","family":"Temple","sequence":"additional","affiliation":[{"name":"Division of Orthopedic Oncology University of Miami Miller School of Medicine  Miami Florida USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brooke","family":"Crawford","sequence":"additional","affiliation":[{"name":"Division of Orthopedic Oncology University of Miami Miller School of Medicine  Miami Florida USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6169-5238","authenticated-orcid":false,"given":"Jonathan","family":"Trent","sequence":"additional","affiliation":[{"name":"Division of Medical Oncology University of Miami Miller School of Medicine  Miami Florida USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0983-8016","authenticated-orcid":false,"given":"Emily E.","family":"Jonczak","sequence":"additional","affiliation":[{"name":"Division of Medical Oncology University of Miami Miller 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