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Title: The second European interdisciplinary Ewing sarcoma research summit--A joint effort to deconstructing the multiple layers of a complex disease.
Authors: Kovar, Heinrich
Amatruda, James
Brunet, Erika
Burdach, Stefan
Cidre-Aranaz, Florencia
de Alava, Enrique
Dirksen, Uta
van der Ent, Wietske
Grohar, Patrick
Grünewald, Thomas G P
Helman, Lee
Houghton, Peter
Iljin, Kristiina
Korsching, Eberhard
Ladanyi, Marc
Lawlor, Elizabeth
Lessnick, Stephen
Ludwig, Joseph
Meltzer, Paul
Metzler, Markus
Mora, Jaume
Moriggl, Richard
Nakamura, Takuro
Papamarkou, Theodore
Radic Sarikas, Branka
Rédini, Francoise
Richter, Guenther H S
Rossig, Claudia
Schadler, Keri
Schäfer, Beat W
Scotlandi, Katia
Sheffield, Nathan C
Shelat, Anang
Snaar-Jagalska, Ewa
Sorensen, Poul
Stegmaier, Kimberly
Stewart, Elizabeth
Sweet-Cordero, Alejandro
Szuhai, Karoly
Tirado, Oscar M
Tirode, Franck
Toretsky, Jeffrey
Tsafou, Kalliopi
Üren, Aykut
Zinovyev, Andrei
Delattre, Olivier
Keywords: Ewing sarcoma;development;epigenetics;microenvironment;therapy
metadata.dc.subject.mesh: Bone Neoplasms
Gene Expression Regulation, Neoplastic
Oncogene Proteins, Fusion
Proto-Oncogene Protein c-fli-1
RNA-Binding Protein EWS
Sarcoma, Ewing
Signal Transduction
Issue Date: 2016
Abstract: Despite multimodal treatment, long term outcome for patients with Ewing sarcoma is still poor. The second "European interdisciplinary Ewing sarcoma research summit" assembled a large group of scientific experts in the field to discuss their latest unpublished findings on the way to the identification of novel therapeutic targets and strategies. Ewing sarcoma is characterized by a quiet genome with presence of an EWSR1-ETS gene rearrangement as the only and defining genetic aberration. RNA-sequencing of recently described Ewing-like sarcomas with variant translocations identified them as biologically distinct diseases. Various presentations adressed mechanisms of EWS-ETS fusion protein activities with a focus on EWS-FLI1. Data were presented shedding light on the molecular underpinnings of genetic permissiveness to this disease uncovering interaction of EWS-FLI1 with recently discovered susceptibility loci. Epigenetic context as a consequence of the interaction between the oncoprotein, cell type, developmental stage, and tissue microenvironment emerged as dominant theme in the discussion of the molecular pathogenesis and inter- and intra-tumor heterogeneity of Ewing sarcoma, and the difficulty to generate animal models faithfully recapitulating the human disease. The problem of preclinical development of biologically targeted therapeutics was discussed and promising perspectives were offered from the study of novel in vitro models. Finally, it was concluded that in order to facilitate rapid pre-clinical and clinical development of novel therapies in Ewing sarcoma, the community needs a platform to maintain knowledge of unpublished results, systems and models used in drug testing and to continue the open dialogue initiated at the first two Ewing sarcoma summits.
metadata.dc.identifier.doi: 10.18632/oncotarget.6937
Appears in Collections:Producción 2020

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