RT Journal Article
T1 The second European interdisciplinary Ewing sarcoma research summit--A joint effort to deconstructing the multiple layers of a complex disease.
A1 Kovar, Heinrich
A1 Amatruda, James
A1 Brunet, Erika
A1 Burdach, Stefan
A1 Cidre-Aranaz, Florencia
A1 de Alava, Enrique
A1 Dirksen, Uta
A1 van der Ent, Wietske
A1 Grohar, Patrick
A1 Grünewald, Thomas G P
A1 Helman, Lee
A1 Houghton, Peter
A1 Iljin, Kristiina
A1 Korsching, Eberhard
A1 Ladanyi, Marc
A1 Lawlor, Elizabeth
A1 Lessnick, Stephen
A1 Ludwig, Joseph
A1 Meltzer, Paul
A1 Metzler, Markus
A1 Mora, Jaume
A1 Moriggl, Richard
A1 Nakamura, Takuro
A1 Papamarkou, Theodore
A1 Radic Sarikas, Branka
A1 Rédini, Francoise
A1 Richter, Guenther H S
A1 Rossig, Claudia
A1 Schadler, Keri
A1 Schäfer, Beat W
A1 Scotlandi, Katia
A1 Sheffield, Nathan C
A1 Shelat, Anang
A1 Snaar-Jagalska, Ewa
A1 Sorensen, Poul
A1 Stegmaier, Kimberly
A1 Stewart, Elizabeth
A1 Sweet-Cordero, Alejandro
A1 Szuhai, Karoly
A1 Tirado, Oscar M
A1 Tirode, Franck
A1 Toretsky, Jeffrey
A1 Tsafou, Kalliopi
A1 Üren, Aykut
A1 Zinovyev, Andrei
A1 Delattre, Olivier
K1 Ewing sarcoma
K1 development
K1 epigenetics
K1 microenvironment
K1 therapy
AB 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Ewing sarcoma research summit" assembled a large group of scientific experts in the field to discuss their latest unpublished findings on the way to the identification of novel therapeutic targets and strategies.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.
YR 2016
FD 2016
LK http://hdl.handle.net/10668/9760
UL http://hdl.handle.net/10668/9760
LA en
DS RISalud
RD Apr 10, 2025