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J Clin Oncol. 2015 Sep 20;33(27):2999-3007. doi: 10.1200/JCO.2015.62.1888. Epub 2015 Aug 24.

Advances in Wilms Tumor Treatment and Biology: Progress Through International Collaboration.

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  • 1Jeffrey S. Dome, Children's National Health System, Washington, DC; James I. Geller, Cincinnati Children's Hospital Medical Center, Cincinnati, OH; Elizabeth A. Mullen, Dana-Farber Cancer Institute, Boston, MA; Norbert Graf, Saarland University, Homburg, Germany; Conrad V. Fernandez, IWK Health Centre, Halifax, Nova Scotia, Canada; Filippo Spreafico, Fondazione Istituto di Ricovero e Cura a Carattere Scientifico, Istituto Nazionale dei Tumori, Milan, Italy; Marry Van den Heuvel-Eibrink, Princess Màxima Center for Pediatric Oncology, Utrecht, the Netherlands; and Kathy Pritchard-Jones, University College London Institute of Child Health, London, United Kingdom. jdome@childrensnational.org.
  • 2Jeffrey S. Dome, Children's National Health System, Washington, DC; James I. Geller, Cincinnati Children's Hospital Medical Center, Cincinnati, OH; Elizabeth A. Mullen, Dana-Farber Cancer Institute, Boston, MA; Norbert Graf, Saarland University, Homburg, Germany; Conrad V. Fernandez, IWK Health Centre, Halifax, Nova Scotia, Canada; Filippo Spreafico, Fondazione Istituto di Ricovero e Cura a Carattere Scientifico, Istituto Nazionale dei Tumori, Milan, Italy; Marry Van den Heuvel-Eibrink, Princess Màxima Center for Pediatric Oncology, Utrecht, the Netherlands; and Kathy Pritchard-Jones, University College London Institute of Child Health, London, United Kingdom.

Abstract

Clinical trials in Wilms tumor (WT) have resulted in overall survival rates of greater than 90%. This achievement is especially remarkable because improvements in disease-specific survival have occurred concurrently with a reduction of therapy for large patient subgroups. However, the outcomes for certain patient subgroups, including those with unfavorable histologic and molecular features, bilateral disease, and recurrent disease, remain well below the benchmark survival rate of 90%. Therapy for WT has been advanced in part by an increasingly complex risk-stratification system based on patient age; tumor stage, histology, and volume; response to chemotherapy; and loss of heterozygosity at chromosomes 1p and 16q. A consequence of this system has been the apportionment of patients into such small subgroups that only collaboration between large international WT study groups will support clinical trials that are sufficiently powered to answer challenging questions that move the field forward. This article gives an overview of the Children's Oncology Group and International Society of Pediatric Oncology approaches to WT and focuses on four subgroups (stage IV, initially inoperable, bilateral, and relapsed WT) for which international collaboration is pressing. In addition, biologic insights resulting from collaborative laboratory research are discussed. A coordinated expansion of international collaboration in both clinical trials and laboratory science will provide real opportunity to improve the treatment and outcomes for children with renal tumors on a global level.

© 2015 by American Society of Clinical Oncology.

PMID:
26304882
[PubMed - indexed for MEDLINE]
PMCID:
PMC4567702
[Available on 2016-09-20]
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