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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

Journal of Clinical Oncology : Official Journal of the American Society of Clinical Oncology
|August 26, 2015
PubMed
Summary
This summary is machine-generated.

Ewing sarcoma (ES) treatment combines chemotherapy, surgery, and radiation. Survival rates for localized disease are 65-75%, but less than 30% for metastatic ES.

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Area of Science:

  • Pediatric Oncology
  • Medical Oncology
  • Sarcoma Research

Background:

  • Ewing sarcoma (ES) is an aggressive bone and soft tissue cancer peaking in adolescents and young adults.
  • Current treatment involves multidisciplinary approaches including chemotherapy, surgery, and radiotherapy.
  • Despite advances, survival rates for metastatic or recurrent ES remain poor.

Purpose of the Study:

  • To review current treatment strategies for Ewing sarcoma.
  • To discuss the efficacy of various chemotherapy regimens and prognostic factors.
  • To explore future therapeutic directions based on ES biology.

Main Methods:

  • Review of national and international clinical trials and treatment optimization efforts.
  • Analysis of first-line chemotherapy trials incorporating multiple drug combinations.
  • Identification and utilization of prognostic factors for tailored therapies.

Main Results:

  • Localized ES 5-year survival is 65-75%; metastatic disease survival is <30% (except isolated pulmonary metastases ~50%).
  • Recurrent ES carries a dismal prognosis.
  • The role of high-dose chemotherapy is still under investigation.

Conclusions:

  • Multidisciplinary treatment has improved outcomes for localized Ewing sarcoma.
  • Significant challenges remain in treating metastatic and recurrent disease.
  • Translating biological insights, particularly regarding EWS-FLI1, into novel therapies is crucial for future progress.