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P. by the University of Zurich and University Hospital of Zurich."},{"DOI":"10.13039\/501100006447","name":"University of Zurich","doi-asserted-by":"crossref","id":[{"id":"10.13039\/501100006447","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Human Cell"],"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Extraskeletal myxoid chondrosarcoma (EMC) is a malignant mesenchymal neoplasm of uncertain differentiation as classified by the WHO Classification of Tumours 2020. Although often associated with pronlonged survival, EMC has high rates of distant recurrences and disease-associated death. EMCs are translocation sarcomas and harbor in\u2009&gt;\u200990% of the cases an <jats:italic>NR4A3<\/jats:italic> rearrangement. The molecular consequences of the <jats:italic>NR4A3<\/jats:italic> gene fusions are not yet fully elucidated as well-characterized ex vivo cell models for EMC are lacking. Patient-derived ex vivo models are important and essential tools for investigating disease mechanisms associated with diseases that are rare, that exhibit poor prognosis and for the identification of potential novel treatment options. We established two novel EMC ex vivo models (<jats:italic>USZ20-EMC1 and USZ22-EMC2<\/jats:italic>) for functional testing and research purposes. <jats:italic>USZ20-EMC1<\/jats:italic> and <jats:italic>USZ22-EMC2<\/jats:italic> were established and maintained as sarco-sphere cell models for several months in culture. The cells were molecularly characterized using DNA sequencing and methylation profiling. Both cell models represent their native tumor tissue as confirmed by histomorphology and their molecular profiles, suggesting that native tumor cell function can be recapitulated in the ex vivo models. Using a functional screening approach, novel anti-cancer drug sensitivities including potential synergistic combinations were identified. In conclusion, two novel EMC ex vivo cell models (<jats:italic>USZ20-EMC1 and USZ22-EMC2<\/jats:italic>) were successfully established and characterized from native tumor tissues. Both cell models will be useful tools for further investigating disease mechanisms and for answering basic and translational research questions.<\/jats:p>","DOI":"10.1007\/s13577-022-00818-x","type":"journal-article","created":{"date-parts":[[2022,11,1]],"date-time":"2022-11-01T00:04:30Z","timestamp":1667261070000},"page":"446-455","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":11,"title":["Establishment, characterization and functional testing of two novel ex vivo extraskeletal myxoid chondrosarcoma (EMC) cell models"],"prefix":"10.1007","volume":"36","author":[{"given":"Jana Lucia","family":"Bangerter","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kim Jannis","family":"Harnisch","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yanjiang","family":"Chen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Catherine","family":"Hagedorn","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lara","family":"Planas-Paz","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9621-8511","authenticated-orcid":false,"given":"Chantal","family":"Pauli","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2022,11,1]]},"reference":[{"key":"818_CR1","doi-asserted-by":"publisher","first-page":"200","DOI":"10.3322\/caac.21605","volume":"70","author":"AC Gamboa","year":"2020","unstructured":"Gamboa AC, Gronchi A, Cardona K. 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No conflict of interest, financial or otherwise, is declared by all the other authors.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflict of interest"}},{"value":"The study was performed following regional\/cantonal and institutional guidelines and in compliance with the Helsinki Declaration and after approval by our cantonal ethical review board Zurich (BASEC-2021-00417).","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethical approval and informed consent"}}]}}