{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,20]],"date-time":"2026-08-20T16:29:01Z","timestamp":1787243341871,"version":"build-2736575974"},"reference-count":46,"publisher":"Journal of Neurosurgery Publishing Group (JNSPG)","issue":"5","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2023,11,1]]},"abstract":"<jats:sec>\n                    <jats:title>OBJECTIVE<\/jats:title>\n                    <jats:p>Chordomas are rare bone neoplasms characterized by a high recurrence rate and no benefit from any approved medical treatment to date. However, the investigation of molecular alterations in chordomas could be essential to prognosticate, guide clinical decision-making, and identify theranostic biomarkers. The aim of this study was to provide a detailed genomic landscape of a homogeneous series of 64 chordoma samples, revealing driver events, theranostic markers, and outcome-related genomic features.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>METHODS<\/jats:title>\n                    <jats:p>The authors conducted whole-exome sequencing (WES), targeted next-generation sequencing, and RNA sequencing of 64 skull base and spinal chordoma samples collected between December 2006 and September 2020. Clinical, histological, and radiological data were retrospectively analyzed and correlated to genetic findings.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>RESULTS<\/jats:title>\n                    <jats:p>\n                      The authors identified homozygous deletions of\n                      <jats:italic>CDKN2A\/2B, PIK3CA<\/jats:italic>\n                      mutations, and alterations affecting genes of SWI\/SNF chromatin remodeling complexes (\n                      <jats:italic>PBRM1<\/jats:italic>\n                      and\n                      <jats:italic>ARID1A<\/jats:italic>\n                      ) as potential theranostic biomarkers. Using matched germline WES, they observed a higher frequency of a common genetic variant (rs2305089; p.(Gly177Asp)) in\n                      <jats:italic>TBXT<\/jats:italic>\n                      (97.8%, p &lt; 0.001) compared to its distribution in the general population.\n                      <jats:italic>PIK3CA<\/jats:italic>\n                      mutation was identified as an independent biomarker of short progression-free survival (HR 10.68, p = 0.0008). Loss of\n                      <jats:italic>CDKN2A\/2B<\/jats:italic>\n                      was more frequently observed in spinal tumors and recurrent tumors.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>CONCLUSIONS<\/jats:title>\n                    <jats:p>\n                      In the current study, the authors identified driver events such as\n                      <jats:italic>PBRM1<\/jats:italic>\n                      and\n                      <jats:italic>PIK3CA<\/jats:italic>\n                      mutations,\n                      <jats:italic>TBXT<\/jats:italic>\n                      alterations, or homozygous deletions of\n                      <jats:italic>CDKN2A\/2B,<\/jats:italic>\n                      which could, for some, be considered potential theranostic markers and could allow for identifying novel therapeutic approaches. With the aim of a future biomolecular prognostication classification, alterations affecting\n                      <jats:italic>PIK3CA<\/jats:italic>\n                      and\n                      <jats:italic>CDKN2A\/2B<\/jats:italic>\n                      could be considered as poor prognostic biomarkers.\n                    <\/jats:p>\n                  <\/jats:sec>","DOI":"10.3171\/2023.1.jns222180","type":"journal-article","created":{"date-parts":[[2023,4,7]],"date-time":"2023-04-07T15:39:45Z","timestamp":1680881985000},"page":"1270-1280","source":"Crossref","is-referenced-by-count":13,"title":["The mutational landscape of skull base and spinal chordomas and the identification of potential prognostic and theranostic biomarkers"],"prefix":"10.3171","volume":"139","author":[{"given":"Thibault","family":"Passeri","sequence":"first","affiliation":[{"name":"Departments of Genetics and"},{"name":"Departments of Neurosurgery,"},{"name":"Department of Translational Research, Laboratory of Preclinical Investigation, Institut Curie, Paris-Saclay University, Paris;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tom","family":"Gutman","sequence":"additional","affiliation":[{"name":"Inserm U900, Institut Curie, Paris;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Abderaouf","family":"Hamza","sequence":"additional","affiliation":[{"name":"Departments of Genetics and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Homa","family":"Adle-Biassette","sequence":"additional","affiliation":[{"name":"Pathology, and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elodie","family":"Girard","sequence":"additional","affiliation":[{"name":"Inserm U900, Institut Curie, Paris;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Romane","family":"Beaurepere","sequence":"additional","affiliation":[{"name":"Departments of Genetics and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zakia","family":"Tariq","sequence":"additional","affiliation":[{"name":"Departments of Genetics and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Odette","family":"Mariani","sequence":"additional","affiliation":[{"name":"Tumor Biology, Institut Curie, Paris;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ahmed","family":"Dahmani","sequence":"additional","affiliation":[{"name":"Department of Translational Research, Laboratory of Preclinical Investigation, Institut Curie, Paris-Saclay University, Paris;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christine","family":"Bourneix","sequence":"additional","affiliation":[{"name":"Departments of Genetics and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rosaria","family":"Abbritti","sequence":"additional","affiliation":[{"name":"Departments of Neurosurgery,"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Keltouma","family":"Driouch","sequence":"additional","affiliation":[{"name":"Departments of Genetics and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Myl\u00e8ne","family":"Bohec","sequence":"additional","affiliation":[{"name":"ICGex NGS Platform, Institut Curie, Paris;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicolas","family":"Servant","sequence":"additional","affiliation":[{"name":"Inserm U900, Institut Curie, Paris;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sylvain","family":"Baulande","sequence":"additional","affiliation":[{"name":"ICGex NGS Platform, Institut Curie, Paris;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Didier","family":"Decaudin","sequence":"additional","affiliation":[{"name":"Department of Translational Research, Laboratory of Preclinical Investigation, Institut Curie, Paris-Saclay University, Paris;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jean-Pierre","family":"Guichard","sequence":"additional","affiliation":[{"name":"Radiology, Lariboisi\u00e8re Hospital, Assistance Publique des H\u00f4pitaux de Paris, University of Paris, Paris;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Valentin","family":"Calugaru","sequence":"additional","affiliation":[{"name":"Department of Radiotherapy, Proton Therapy Center, Institut Curie, Paris-Saclay University, Orsay;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lo\u00efc","family":"Feuvret","sequence":"additional","affiliation":[{"name":"Department of Radiotherapy, Piti\u00e9-Salp\u00eatri\u00e8re Hospital, Assistance Publique des H\u00f4pitaux de Paris, Paris;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jean-Marc","family":"Guinebreti\u00e8re","sequence":"additional","affiliation":[{"name":"Department of Pathology, Institut Curie, Paris-Saclay University, Paris; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laurence","family":"Champion","sequence":"additional","affiliation":[{"name":"Department of Nuclear Medicine, Institut Curie, Paris-Saclay University, Saint-Cloud, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ivan","family":"Bi\u00e8che","sequence":"additional","affiliation":[{"name":"Departments of Genetics and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S\u00e9bastien","family":"Froelich","sequence":"additional","affiliation":[{"name":"Departments of Neurosurgery,"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hamid","family":"Mammar","sequence":"additional","affiliation":[{"name":"Department of Radiotherapy, Proton Therapy Center, Institut Curie, Paris-Saclay University, Orsay;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Julien","family":"Masliah-Planchon","sequence":"additional","affiliation":[{"name":"Departments of Genetics and"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1754","reference":[{"issue":"4","key":"b1","series-title":"J Neurosurg Spine","first-page":"422","article-title":"A historical recount of chordoma","volume":"28","author":"Sahyouni R","year":"2018","unstructured":"Sahyouni R, Goshtasbi K, Mahmoodi A, Chen JW. 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