Journal
JOURNAL OF CLINICAL ONCOLOGY
Volume 33, Issue 27, Pages 2999-U94Publisher
AMER SOC CLINICAL ONCOLOGY
DOI: 10.1200/JCO.2015.62.1888
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Categories
Funding
- National Institutes of Health [U10CA098543]
- Children's Oncology Group [U10CA098543, U10CA180886, U10CA180899, U10CA098413]
- European Union [261474, 270089]
- P-Medicine: From Data Sharing and Integration via VPH Models [270089]
- Cancer Research United Kingdom [C1188/A4614]
- Great Ormond Street Hospital Children's Charity
- Children with Cancer
- National Institutes of Health Research Great Ormond Street Hospital-University College London Biomedical Research Centre
- German Cancer Aid for International Society of Pediatric Oncology Gesellschaft fur Padiatrische Onkologie und Hamatologie [50-2709-Gr2]
- Cancer Research UK [17297] Funding Source: researchfish
- Great Ormond Street Hospital Childrens Charity [W1090] Funding Source: researchfish
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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. (C) 2015 by American Society of Clinical Oncology
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