Contributors: Children’s Cancer Research Institute Vienna, Austria; Department of Pediatrics Vienna, Austria; Medizinische Universität Wien = Medical University of Vienna; Departments of Pediatrics, Molecular Biology and Internal Medicine Dallas, TX, USA; University of Texas Southwestern Medical Center Dallas; Structure et Instabilité des Génomes (STRING); Muséum national d'Histoire naturelle (MNHN)-Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut de Chimie - CNRS Chimie (INC-CNRS)-Centre National de la Recherche Scientifique (CNRS); Klinikum rechts der Isar (MRI TUM); Technische Universität Munchen - Technical University Munich - Université Technique de Munich (TUM); Unidad de Tumores Sólidos Infantiles – Unidad de Investigación Biomédica Madrid, Spain; Instituto de Salud Carlos III Madrid (ISC)-Instituto de Investigación en Enfermedades Raras (IIER); Laboratorio de Patología Molecular (USAL-CSIC); Centro de Investigación del Cáncer; Universitätsklinikum Münster Munster, Germany; Unité de génétique et biologie des cancers (U830); Université Paris Descartes - Paris 5 (UPD5)-Institut Curie Paris -Institut National de la Santé et de la Recherche Médicale (INSERM); Institute of Biology Leiden, The Netherlands; Universiteit Leiden = Leiden University; Center for Cancer and Cell Biology Grand Rapids, MI, USA; Van Andel Institute Grand Rapids -Helen DeVos Children’s Hospital Grand Rapids, MI, USA; Laboratory for Pediatric Sarcoma Biology Munich, Germany; Ludwig-Maximilian-Universität München Pathologisches Institut Germany; Center for Cancer Rearch Bethesda, MA, USA; National Cancer Institute Bethesda (NCI-NIH); National Institutes of Health Bethesda, MD, USA (NIH)-National Institutes of Health Bethesda, MD, USA (NIH); Greehey Children’s Cancer Research Institute San Antonio, TX, USA; The University of Texas Health Science Center at Houston (UTHealth); VTT Technical Research Centre of Finland (VTT); Institute of Bioinformatics Muenster, Germany; Westfälische Wilhelms-Universität Münster = University of Münster (WWU); Department of Pathology and Human Oncology and Pathogenesis Program New York, NY, USA; Memorial Sloane Kettering Cancer Center New York; Department of Pediatrics and Department of Pathology Ann Arbor, MI, USA; University of Michigan Ann Arbor; University of Michigan System-University of Michigan System; Division of Pediatric Hematology/ Oncology/BMT Columbus, OH, USA; Abigail Wexner Research Institute; Nationwide Children's Hospital-Nationwide Children's Hospital; Department of Sarcoma Medical Oncology Houston, TX, USA; The University of Texas M.D. Anderson Cancer Center Houston; Genetics Branch Bethesda, MD, USA (Center for Cancer Research); Pediatric Oncology and Hematology Erlangen, Germany; University Hospital Erlangen = Uniklinikum Erlangen; Department of Pediatric Oncology Barcelona, Spain; Hospital Sant Joan de Déu Barcelona; Ludwig Boltzmann Institute for Cancer Research Vienna, Austria; Institute of Animal Breeding and Genetics Vienna, Austria (Department for Biomedical Sciences); University of Veterinary Medicine Vienna (Vetmeduni); Division of Carcinogenesis Tokyo, Japan; The Cancer Institute Tokyo, Japan -Japanese Foundation for Cancer Research Tokyo, Japan; School of Mathematics and Statistics Glasgow, UK; University of Glasgow; Research Center for Molecular Medicine of the Austrian Academy of Sciences Vienna, Austria (CeMM ); Austrian Academy of Sciences (OeAW); Physiopathologie des Adaptations Nutritionnelles (PhAN); Université de Nantes - UFR de Médecine et des Techniques Médicales (UFR MEDECINE); Université de Nantes (UN)-Université de Nantes (UN)-Institut National de Recherche pour l’Agriculture, l’Alimentation et l’Environnement (INRAE); Department of Pediatrics Research Houston, TX, USA; Universität Zürich Zürich = University of Zurich (UZH); CRS Development of Biomolecular Therapies Bologna, Italy (Experimental Oncology Lab); Alma Mater Studiorum Università di Bologna = University of Bologna (UNIBO)-The Rizzoli Institute Bologna, Italy; Department of Chemical Biology and Therapeutics Memphis,TN, USA; St Jude Children's Research Hospital; Department of Molecular Oncology British Columbia, Canada; British Columbia Cancer Research Centre British Columbia, Canada; Department of Pediatric Oncology Boston, MA, USA; Dana-Farber Cancer Institute Boston; Department of Developmental Neurobiology Memphis, TN, USA; Division of Hematology and Oncology Stanford, CA, USA (Department of Pediatrics); Stanford University; Department of Molecular Cell Biology Leiden, The Netherlands; Leiden University Medical Center (LUMC); Universiteit Leiden = Leiden University -Universiteit Leiden = Leiden University; Molecular Oncology Laboratory Barcelona, Spain (Sarcoma Research Group); Institut d'Investigació Biomèdica de Bellvitge = Bellvitge Biomedical Research Institute (IDIBELL)-L’Hospitalet de Llobregat Barcelona, Spain; Department of Oncology Washington, DC, USA; Washington University School of Medicine in St. Louis; Washington University in Saint Louis (WUSTL)-Washington University in Saint Louis (WUSTL); Cancer et génome: Bioinformatique, biostatistiques et épidémiologie d'un système complexe; Mines Paris - PSL (École nationale supérieure des mines de Paris); Université Paris Sciences et Lettres (PSL)-Université Paris Sciences et Lettres (PSL)-Institut Curie Paris -Institut National de la Santé et de la Recherche Médicale (INSERM); The conference was supported by the European Union’s Seventh Framework Programme grants 261743 (ENCCA) and 259348 (ASSET).; European Project: 259348,EC:FP7:HEALTH,FP7-HEALTH-2010-two-stage,ASSET(2010)
نبذة مختصرة : International audience ; 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.
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