4. Wilms tumor

  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. Ananth Shankar

Published Online: 8 MAY 2013

DOI: 10.1002/9781118625309.ch4

Evidence-Based Pediatric Oncology

Evidence-Based Pediatric Oncology

How to Cite

Shankar, A. (2013) Wilms tumor, in Evidence-Based Pediatric Oncology (eds R. Pinkerton, A. Shankar and K. K. Matthay), John Wiley & Sons, Ltd, Oxford. doi: 10.1002/9781118625309.ch4

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. University College London Hospitals NHS Foundation Trust, London, UK

Publication History

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

ISBN Information

Print ISBN: 9780470659649

Online ISBN: 9781118625309

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Keywords:

  • children;
  • nephrectomy;
  • radiotherapy;
  • renal tumors;
  • Wilms tumor

Summary

This chapter summarizes the huge progress that has been made since the early 1970s in the treatment of Wilms tumor and other renal tumors of childhood. The first two trials of the North American National Wilms Tumor Study Group (NWTSG) showed that in patients whose tumors were amenable to immediate nephrectomy and whose treatment was carefully controlled, relapse-free survival rates of over 80% could be achieved. Ever since, the majority of children with Wilms tumor have been offered entry into randomized trials that have sought to reduce both the duration and intensity of their therapy without compromising relapse-free survival. These have been consistently successful, to the extent that for children treated in the 1990s, a decreasing proportion were treated with radiotherapy or doxorubicin whilst event-free and overall survival continued to improve. This emphasizes the importance of randomized trials rather than relying on historical comparisons.