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Ewing Sarcoma: Current Management and Future Approaches Through Collaboration
Nathalie Gaspar1, Douglas S Hawkins1, Uta Dirksen1
1Nathalie Gaspar, Marie-Cecile Le Deley, and Odile Oberlin, Institut Gustave Roussy, Villejuif; Nathalie Gaspar, Marie-Cecile Le Deley, Line Claude, Olivier Delattre, Jean Michon, Perrine Marec-Bérard, and Odile Oberlin, Société Française de Lutte Contre les Cancers et les Leucémies de l'Enfant et de l'Adolescent; Marie-Cecile Le Deley, Paris-Sud University, Le Kremlin-Bicêtre; Line Claude and Perrine Marec-Bérard, Centre Léon-Bérard, Lyon; Olivier Delattre and Jean Michon, Institut Curie, Paris, France; Douglas S. Hawkins, Seattle Children's Hospital, Seattle, WA; Douglas S. Hawkins, Mark L. Bernstein, and Richard B. Womer, Children's Oncology Group; Uta Dirksen and Heribert Juergens, University Hospital Münster, Münster; Uta Dirksen, Michael Paulussen, and Heribert Juergens, Gesellschaft für Pädiatrische Onkologie und Hämatologie; Michael Paulussen, Children's and Adolescents' Hospital, Witten/Herdecke University, Datteln, Germany; Ian J. Lewis, Alder Hey Children's National Health Service (NHS) Foundation Trust, Liverpool; Ian J. Lewis, Robert Grimer, Bernadette Brennan, and Alan W. Craft, Children's Cancer and Leukaemia Group; Robert Grimer, Royal Orthopaedic Hospital, Birmingham; Jeremy Whelan, University College London Hospital NHS Foundation Trust; Ian Judson, Royal Marsden Hospital, London; Bernadette Brennan, Royal Manchester Children's Hospital, Manchester; Alan W. Craft, Royal Victoria Infirmary, Newcastle, United Kingdom; Stefano Ferrari and Piero Picci, Rizzoli Institute, Bologna; Stefano Ferrari, Piero Picci, Roberto Luksch, Italian Sarcoma Group; Roberto Luksch, Fondazione Istituto di Ricovero e Cura a Carattere Scientifico Istituto Nazionale dei Tumori, Milan, Italy; Heinrich Kovar and Ruth Ladenstein, Children's Cancer Institute, St Anna Kinderkrebsforschung, Wien; Heinrich Kovar and Ruth Ladenstein, Arbeitsgemeinschaft Ambulant Tätiger Pädiatrischer Onkologen und Hämatologen, Vienna, Austria; Jeremy Whelan and Ian Judson, European O
Abstract:
Ewing sarcoma (ES) is an aggressive sarcoma of bone and soft tissue occurring at any age with a peak incidence in adolescents and young adults. The treatment of ES relies on a multidisciplinary approach, coupling risk-adapted intensive neoadjuvant and adjuvant chemotherapies with surgery and/or radiotherapy for control of the primary site and possible metastatic disease. The optimization of ES multimodality therapeutic strategies has resulted from the efforts of several national and international groups in Europe and North America and from cooperation between pediatric and medical oncologists. Successive first-line trials addressed the efficacy of various cyclic combinations of drugs incorporating doxorubicin, vincristine, cyclophosphamide, ifosfamide, etoposide, and dactinomycin and identified prognostic factors now used to tailor therapies. The role of high-dose chemotherapy is still debated. Current 5-year overall survival for patients with localized disease is 65% to 75%. Patients with metastases have a 5-year overall survival < 30%, except for those with isolated pulmonary metastasis (approximately 50%). Patients with recurrence have a dismal prognosis. The many insights into the biology of the EWS-FLI1 protein in the initiation and progression of ES remain to be translated into novel therapeutic strategies. Current options and future approaches will be discussed.
