3. Ewing sarcoma

  1. Ross Pinkerton MB, BCh, BaO, MD Executive Director, Division of Oncology2,
  2. Ananth Shankar MD, FRCPCH Consultant in Paediatric and Adolescent Oncology3 and
  3. Katherine K. Matthay BA, MD Mildred V. Strouss Professor of Translational Oncology, Director, Pediatric Hematology-Oncology4
  1. Katherine K. Matthay

Published Online: 8 MAY 2013

DOI: 10.1002/9781118625309.ch3

Evidence-Based Pediatric Oncology

Evidence-Based Pediatric Oncology

How to Cite

Matthay, K. K. (2013) Ewing sarcoma, in Evidence-Based Pediatric Oncology (eds R. Pinkerton, A. Shankar and K. K. Matthay), John Wiley & Sons, Ltd, Oxford. doi: 10.1002/9781118625309.ch3

Editor Information

  1. 2

    Royal Children's Hospital, Children's Health Queensland, Brisbane, QLD, Australia

  2. 3

    University College London Hospitals NHS Foundation Trust London, UK

  3. 4

    Department of Pediatrics, UCSF School of Medicine and, UCSF Benioff Children's Hospital, San Francisco, CA, USA

Author Information

  1. UCSF School of Medicine, San Francisco, CA, USA

Publication History

  1. Published Online: 8 MAY 2013
  2. Published Print: 20 MAY 2013

ISBN Information

Print ISBN: 9780470659649

Online ISBN: 9781118625309



  • chemotherapy;
  • children;
  • clinical trials;
  • Ewing sarcoma;
  • oncology


Outcomes for patients with localized Ewing sarcoma have improved dramatically over the past three decades. In North America, INT-0091 established a new standard of care for patients with localized Ewing sarcoma consisting of vincristine/doxorubicin/cyclophosphamide alternating with ifosfamide/etoposide. The current Children's Oncology Group (COG) trial for patients with localized Ewing sarcoma seeks to intensify therapy by adding another active chemotherapy combination to standard therapy. In Europe, early co-operative group clinical trials also demonstrated the activity of regimens that include vincristine, doxorubicin, dactinomycin, and cyclo-phosphamide (VACA). More recent European trials have established other treatment regimens that result in similar outcomes to those reported in North American studies. Of note, the strategy of interval compression has not yet been evaluated in patients with metastatic Ewing sarcoma. Another series of studies have investigated high-dose therapy for patients with poor-risk Ewing sarcoma, most notably newly diagnosed metastatic disease.