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Onc."],"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Arcagen (NCT02834884) is a European prospective study aiming at defining the molecular landscape of rare cancers for treatment guidance. We present data from the cohort of rare thoracic tumors. Patients with advanced pleural mesothelioma (PM) or thymic epithelial tumors (TET) underwent genomic profiling with large targeted assay [&gt;300 genes, tumor mutational burden (TMB), microsatellite instability (MSI) status] on formalin-fixed paraffin-embedded (FFPE) or plasma samples. EORTC molecular tumor board (MTB) advised for biomarker-guided treatments. 102 patients recruited from 8 countries between July 2019 and May 2022 were evaluable: 56 with PM, 46 with TET (23 thymomas, 23 thymic carcinomas). Molecular profiling was performed on 70 FFPE samples (42 PM, 28 TET), and 32 cases on ctDNA (14 PM, 18 TET), within a median turnaround time of 8 days from sample reception. We detected relevant molecular alterations in 66 out of 102 patients (65%; 79% PM, 48% TET), 51 of 70 FFPE samples (73%; 90% PM, 46% TET), and 15 of 32 plasma samples (47%; 43% PM, 50% TET). The most frequently altered genes were <jats:italic>CDKN2A\/B, BAP1, MTAP<\/jats:italic> in PM and <jats:italic>TP53, CDKN2A\/B, SETD2<\/jats:italic> in TET. The TMB was low (mean 3.2 Muts\/MB), 2 PM had MSI-high status. MTB advised molecular-guided treatment options in 32 situations, for 17 PM and 15 TET patients (75% clinical trial option, 22% off-label drug or compassionate use, 3% early access program). Molecular testing and MTB discussion were feasible for patients with rare thoracic cancers and allowed the broadening of treatment options for 30% of the cases.<\/jats:p>","DOI":"10.1038\/s41698-024-00518-9","type":"journal-article","created":{"date-parts":[[2024,2,16]],"date-time":"2024-02-16T12:02:40Z","timestamp":1708084960000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":15,"title":["EORTC-SPECTA Arcagen study, comprehensive genomic profiling and treatment adaptation of rare thoracic cancers"],"prefix":"10.1038","volume":"8","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7461-023X","authenticated-orcid":false,"given":"Marco","family":"Tagliamento","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marie","family":"Morfouace","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Charalambos","family":"Loizides","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Julio","family":"Oliveira","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5807-9503","authenticated-orcid":false,"given":"Laurent","family":"Greillier","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Judith","family":"Raimbourg","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anne-Claire","family":"Toffart","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thierry","family":"Chatellier","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicolas","family":"Cloarec","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0434-3436","authenticated-orcid":false,"given":"Ivana","family":"Sullivan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Birute","family":"Brasiuniene","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael","family":"Duruisseaux","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kersti","family":"Oselin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marie-Sophie","family":"Robert","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carolina","family":"Fernandes","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Arnaud","family":"Poncin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7190-120X","authenticated-orcid":false,"given":"Jean-Yves","family":"Blay","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Benjamin","family":"Besse","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicolas","family":"Girard","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2024,2,16]]},"reference":[{"key":"518_CR1","doi-asserted-by":"publisher","first-page":"516","DOI":"10.21037\/tlcr.2018.10.01","volume":"7","author":"V Nuyts","year":"2018","unstructured":"Nuyts, V., Nawrot, T., Nemery, B. & Nackaerts, K. 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Hansen Fellowship Grant by the International Lung Cancer Foundation; he declared honoraria for activity as medical writer by Amgen, MDS, and support for travel and accommodation expensed by Eli Lilly. Morfouace M is currently Employee at Merck Healthcare. Greillier L declared institutional research grants by Astra Zeneca, BMS, MSD, Takeda, Pfizer, Roche, Amgen, Sanofi, Janssen, Eli Lilly, Novartis, and personal honoraria for lectures, presentations, speakers bureaus from Astra Zeneca, BMS, MSD, Takeda, Pfizer, Roche, Amgen, Sanofi, Janssen, Eli Lilly, Novartis, and support for attending meetings and\/or travel by Pfizer, MSD, Astra Zeneca, Takeda. Raimbourg J declared payment or honoraria for lectures, presentations, speakers bureaus from Pierre Fabre, Sanofi, Ipsen, Amgen, Takeda, and support for attending meetings and\/or travel by Pierre Fabre, Daiichi Sankyo. Toffart AC declared payment or honoraria for lectures, presentations, speakers bureaus from Astra Zeneca, BMS, MSD, Sanofi, and payment for expert testimony from AGIR\u00e0dom, AMGEN, Astra Zeneca, BMS, IPSEN, Janssen, Pfizer, Roche, Sanofi, Takeda, and Support for attending meetings and\/or travel by Astra Zeneca, Roche, Takeda. Sullivan I declared payment or honoraria for lectures, presentations, speakers bureaus from Roche, Takeda, AstraZeneca, Bristol-Myers Squibb, Novartis, Pfizer, Sanofi, and support for attending meetings and\/or travel by Roche, Takeda, Eli Lilly, AstraZeneca, and participation on a Data Safety Monitoring Board or Advisory Board organized by Roche, Novartis, Takeda, Sanofi, Bristol-Myers Squibb. Besse B declared Sponsored Research at Gustave Roussy Cancer Center, Investigator or co-investigator of trials of Abbvie, Amgen, AstraZeneca, Biogen, Blueprint Medicines, BMS, Celgene, Eli Lilly, GSK, Ignyta, IPSEN, Merck KGaA, MSD, Nektar, Onxeo, Pfizer, Pharma Mar, Sanofi, Spectrum Pharmaceuticals, Takeda, Tiziana Pharma, Nerviano, GSK, Pfizer, Roche-Genentech, Lilly, OSE Pharma, MSD, Celgene, Stemcentrx, Ignyta, Abbvie, Loxo Oncology, AstraZeneca, Blueprint Medicines. Girard N declared research grants by AstraZeneca, Amgen, Boehringer Ingelheim, Eli Lilly, Hoffmann-La Roche, Janssen, Merck, Merck Sharp & Dohme, Novartis, Pfizer, Sivan, Trizell, Consultative services for Bristol Myers Squibb, AstraZeneca, AbbVie, Amgen, Boehringer Ingelheim, Eli Lilly, Hoffmann- La Roche, Janssen, Merck, Merck Sharp & Dohme, Mirati, Novartis, Pfizer, Roche, Sanofi, Sivan, Payment for expert testimony from AstraZeneca, and Participation on a data safety monitoring board of Roche, and Leadership role in the Thymic Malignancy Interest Group, and Employment of a family member in AstraZeneca. The other authors declared no COI.","order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"37"}}