The second European interdisciplinary Ewing sarcoma research summit--A joint effort to deconstructing the multiple layers of a complex disease

Kovar H, Amatruda J, Brunet E, Burdach S, Cidre-Aranaz F, De Alava E, Dirksen U, Van Der Ent W, Grohar P, Grunewald TGP, Helman L, Houghton P, Iljin K, Korsching E, Ladanyi M, Lawlor E, Lessnick S, Ludwig J, Meltzer P, Metzler M, Mora J, Moriggl R, Nakamura T, Papamarkou T, Sarikas BR, Redini F, Richter GHS, Rossig C, Schadler K, Schaefer BW, Scotlandi K, Sheffield NC, Shelat A, Snaar-Jagalska E, Sorensen P, Stegmaier K, Stewart E, Sweet-Cordero A, Szuhai K, Tirado OM, Tirode F, Toretsky J, Tsafou K, Uren A, Zinovyev A, Delattre O (2016)

Publication Type: Journal article

Publication year: 2016

Journal

Book Volume: 7

Pages Range: 8613-24

Journal Issue: 8

DOI: 10.18632/oncotarget.6937

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Ewing sarcoma research summit" assembled a large group of scientific experts in the field to discuss their latest unpublished findings on the way to the identification of novel therapeutic targets and strategies.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.

Authors with CRIS profile

Involved external institutions

St. Anna Kinderkrebsforschung Austria (AT) University of Texas Southwestern Medical Center (UT Southwestern) United States (USA) (US) Muséum National d’Histoire Naturelle (MNHN) France (FR) Technische Universität München (TUM) Germany (DE) Universidad Carlos III de Madrid (UC3M) Spain (ES) University of Seville / Universidad de Sevilla Spain (ES) Universitätsklinikum Münster Germany (DE) Institut Curie France (FR) Van Andel Institute United States (USA) (US) Ludwig-Maximilians-Universität (LMU) Germany (DE) National Cancer Institute (NCI) United States (USA) (US) University of Texas Health Science Center at San Antonio (UTHSCSA) United States (USA) (US) VTT Technical Research Centre of Finland Finland (FI) Westfälische Wilhelms-Universität (WWU) Münster Germany (DE) Memorial Sloan Kettering Cancer Center United States (USA) (US) University of Michigan United States (USA) (US) Ohio State University United States (USA) (US) University of Texas MD Anderson Cancer Center United States (USA) (US) Hospital Sant Joan de Déu Barcelona Spain (ES) Ludwig Boltzmann Gesellschaft Austria (AT) Japanese Foundation for Cancer Research Japan (JP) University of Glasgow United Kingdom (GB) Österreichische Akademie der Wissenschaften Austria (AT) Université de Nantes France (FR) Universitäts-Kinderspital Zürich Switzerland (CH) Rizzoli Orthopaedic Institute / Istituto Ortopedico Rizzoli (IRCCS) Italy (IT) St. Jude Children’s Research Hospital United States (USA) (US) Leiden University Netherlands (NL) British Columbia Cancer Agency Canada (CA) Dana–Farber Cancer Institute United States (USA) (US) Stanford University United States (USA) (US) Bellvitge Biomedical Research Institute (IDIBELL) Spain (ES) Georgetown University United States (USA) (US)

How to cite

APA:

Kovar, H., Amatruda, J., Brunet, E., Burdach, S., Cidre-Aranaz, F., De Alava, E.,... Delattre, O. (2016). The second European interdisciplinary Ewing sarcoma research summit--A joint effort to deconstructing the multiple layers of a complex disease. Oncotarget, 7(8), 8613-24. https://doi.org/10.18632/oncotarget.6937

MLA:

Kovar, Heinrich, et al. "The second European interdisciplinary Ewing sarcoma research summit--A joint effort to deconstructing the multiple layers of a complex disease." Oncotarget 7.8 (2016): 8613-24.

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