Abstract
Ewing sarcoma (EWS) is a rare malignant pediatric tumor and patient derived xenografts (PDXs) could represent a possibility to increase the number of available models to study this disease. Compared to cell derived xenografts (CDX), PDXs are reported to better recapitulate tumor microenvironment, heterogeneity, genetic and epigenetic features and are considered reliable models for their better predictive value when comparing preclinical efficacy and treatment response in patients. In this chapter, we extensively describe a method for generating Ewing sarcoma PDX models, for their validation and molecular characterization.
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Acknowledgments
This work is supported by grants received from the Institut Curie; the INSERM; the Canceropôle Ile-de-France; the Ligue Nationale Contre le Cancer (Equipe labellisée) and projet de Recherche “Enfants, Adolescents et Cancer”; the Institut National du Cancer (PLBIO16-291), the Fondation ARC, the Agence Nationale de la Recherche (ANR-10-EQPX-03, Institut Curie Génomique d’Excellence (ICGex) and the société française de lutte contre les cancers de l’enfant et de l’adolescent. The European Union (ERANET TRANSCAN-2_TORPEDO ER-2015-2360405, to KS, TRANSCAN-2_BRCAddict TRANS-201801292 to DS and KS), H2020-lMI2-JTl-201 5-07 (116064—ITCC P4 to KS and DS). DS is supported by SiRIC (Grant « INCa-DGOS-4654).
We thank Cristina Ghinelli for the graphic support and Dr. Marianna Carrabotta (IRCCS-Istituto Ortopedico Rizzoli) for her technical support with evaluation of the EWSR1-ETS fusion transcript. We also thank all parents, patients, and families that consented to provide samples to establish these models. The materials presented and views expressed here are the responsibility of the authors only. The sponsor takes no responsibility for any use made of the information set out.
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Surdez, D., Landuzzi, L., Scotlandi, K., Manara, M.C. (2021). Ewing Sarcoma PDX Models. In: Cidre-Aranaz, F., G. P. Grünewald, T. (eds) Ewing Sarcoma . Methods in Molecular Biology, vol 2226. Humana, New York, NY. https://doi.org/10.1007/978-1-0716-1020-6_18
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DOI: https://doi.org/10.1007/978-1-0716-1020-6_18
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