The second European interdisciplinary Ewing sarcoma research summit - A joint effort to deconstructing the multiple layers of a complex disease
Por:
Kovar H, Amatruda J, Brunet E, Burdach S, Cidre-Aranaz F, de Alava E, Dirksen U, van der Ent W, Grohar P, Grünewald TG, 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, Radic Sarikas B, Rédini F, Richter GH, Rossig C, Schadler K, Schäfer 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, Üren A, Zinovyev A and Delattre O
Publicada:
23 feb 2016
Categoría:
Oncology
Resumen:
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Ewing sarcoma research summit" assembled a large group of scientific experts in the field to discuss their latest unpublished findings on the way to the identification of novel therapeutic targets and strategies.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 intratumor 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and clinical development of novel therapies in Ewing sarcoma, the community needs a platform to maintain knowledge of unpublished results, systems and models used in drug testing and to continue the open dialogue initiated at the first two Ewing sarcoma summits.
Filiaciones:
Kovar H:
Children's Cancer Research Institute, St. Anna Kinderkrebsforschung, Vienna, Austria
Department of Pediatrics, Medical University Vienna, Vienna, Austria
Amatruda J:
Departments of Pediatrics, Molecular Biology and Internal Medicine, University of Texas Southwestern Medical Center, Dallas, TX, USA
Brunet E:
Museum National d'Histoire Naturelle, INSERM U1154, CNRS 7196, Paris, France
Burdach S:
Children's Cancer Research Center and Department of Pediatrics, Klinikum rechts der Isar, Technical University and Comprehensive Cancer Center Munich (CCCM), Munich, Germany
Cidre-Aranaz F:
Unidad de Tumores Sólidos Infantiles, Área de Genética Humana, Instituto de Investigación de Enfermedades Raras, Instituto de Salud Carlos III, Madrid, Spain
de Alava E:
Institute of Biomedicine of Sevilla (IBiS), Virgen del Rocio University Hospital /CSIC/University de Sevilla, Department of Pathology, Seville, Spain
Dirksen U:
University Children´s Hospital Muenster, Pediatric Hematology and Oncology, Muenster, Germany
van der Ent W:
INSERM U830, Laboratoire de Génétique et Biologie des Cancers, Institut Curie, Paris, France
Institute of Biology, Leiden University, Leiden, The Netherlands
Grohar P:
Van Andel Institute, Center for Cancer and Cell Biology and Helen DeVos Children's Hospital, Grand Rapids, MI, USA
Grünewald TG:
Laboratory for Pediatric Sarcoma Biology, Institute of Pathology of the LMU Munich, Munich, Germany
Helman L:
Center for Cancer Rearch, NCI, NIH, Bethesda, MA, USA
Houghton P:
Greehey Children's Cancer Research Institute, University of Texas Health Science Center, San Antonio, TX, USA
Iljin K:
VTT Technical Research Centre of Finland Ltd, Espoo, Finland
Korsching E:
Institute of Bioinformatics, Faculty of Medicine, University of Muenster, Muenster, Germany
Ladanyi M:
Department of Pathology and Human Oncology and Pathogenesis Program, Memorial Sloan-Kettering Cancer Center, New York, NY, USA
Lawlor E:
Department of Pediatrics and Department of Pathology, University of Michigan, Ann Arbor, MI, USA
Lessnick S:
Center for Childhood Cancer and Blood Disorders, Nationwide Children's Hospital, and the Division of Pediatric Hematology/Oncology/BMT, The Ohio State University, Columbus, OH, USA
Ludwig J:
Department of Sarcoma Medical Oncology, MD Anderson Cancer Center, Houston, TX, USA
Meltzer P:
Genetics Branch, Center for Cancer Research, National Cancer Institute, Bethesda, MD, USA
Metzler M:
Pediatric Oncology and Hematology, University Hospital Erlangen, Erlangen, Germany
Mora J:
Department of Pediatric Oncology, Sant Joan de Déu Hospital, Barcelona, Spain
Moriggl R:
Ludwig Boltzmann Institute for Cancer Research, Vienna, Austria
Institute of Animal Breeding and Genetics, University of Veterinary Medicine and Medical University, Vienna, Austria
Nakamura T:
Division of Carcinogenesis, The Cancer Institute, Japanese Foundation for Cancer Research, Tokyo, Japan
Papamarkou T:
University of Glasgow, School of Mathematics and Statistics, Glasgow, UK
Radic Sarikas B:
CeMM Research Center for Molecular Medicine of the Austrian Academy of Sciences, Vienna, Austria
Rédini F:
INSERM UMR957, Université de Nantes, Nantes, France
Richter GH:
Children's Cancer Research Center and Department of Pediatrics, Klinikum rechts der Isar, Technical University and Comprehensive Cancer Center Munich (CCCM), Munich, Germany
Rossig C:
University Children´s Hospital Muenster, Pediatric Hematology and Oncology, Muenster, Germany
Schadler K:
Department of Pediatrics Research, MD Anderson Cancer Center, Houston, TX, USA
Schäfer BW:
Department of Oncology and Children's Research Center, University Children's Hospital, Zurich, Switzerland
Scotlandi K:
CRS Development of Biomolecular Therapies, Experimental Oncology Lab, Rizzoli Institute, Bologna, Italy
Sheffield NC:
CeMM Research Center for Molecular Medicine of the Austrian Academy of Sciences, Vienna, Austria
Shelat A:
Department of Chemical Biology and Therapeutics, St. Jude Children's Research Hospital, Memphis,TN, USA
Snaar-Jagalska E:
Institute of Biology, Leiden University, Leiden, The Netherlands
Sorensen P:
Department of Molecular Oncology, British Columbia Cancer Research Centre, Vancouver, British Columbia, Canada
Stegmaier K:
Department of Pediatric Oncology, Dana-Farber Cancer Institute and Boston Children's Hospital, Boston, MA, USA
Stewart E:
Department of Developmental Neurobiology, St. Jude Children's Research Hospital, Memphis, TN, USA
Sweet-Cordero A:
Division of Hematology and Oncology, Department of Pediatrics, Stanford University, Stanford, CA, USA
Szuhai K:
Department of Molecular Cell Biology, Leiden University Medical Center, Leiden, The Netherlands
Tirado OM:
Sarcoma Research Group, Molecular Oncology Laboratory, Bellvitge Biomedical Research Institute (IDIBELL), L'Hospitalet de Llobregat, Barcelona, Spain
Tirode F:
INSERM U830, Laboratoire de Génétique et Biologie des Cancers, Institut Curie, Paris, France
Toretsky J:
Department of Oncology, Georgetown University School of Medicine, Washington, DC, USA
Tsafou K:
Department of Oncology, Georgetown University School of Medicine, Washington, DC, USA
Üren A:
Department of Oncology, Georgetown University School of Medicine, Washington, DC, USA
Zinovyev A:
INSERM U830, Laboratoire de Génétique et Biologie des Cancers, Institut Curie, Paris, France
INSERM, U900, Paris, France
Ecole des Mines ParisTech, Fontainbleau, France
Delattre O:
INSERM U830, Laboratoire de Génétique et Biologie des Cancers, Institut Curie, Paris, France
Green Submitted, Green Published, gold
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