Long-term outcomes and patterns of relapse in patients with bilateral Wilms tumor or bilaterally predisposed unilateral Wilms tumor, a report from the COG AREN0534 study

Andrew J. Murphy, St Jude Children's Research Hospital, Memphis, Tennessee, USA.
Jack Brzezinski, Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.
Lindsay A. Renfro, Division of Biostatistics, University of Southern California and Children's Oncology Group, Los Angeles, California, USA.
Brett Tornwall, Division of Biostatistics, University of Southern California and Children's Oncology Group, Los Angeles, California, USA.
Marcus M. Malek, Children's Hospital of Pittsburgh, University of Pittsburgh, Pittsburgh, Pennsylvania, USA.
Daniel J. Benedetti, Monroe Carell Jr. Children's Hospital at Vanderbilt, Vanderbilt University Medical Center, Nashville, Tennessee, USA.
Nicholas G. Cost, The Surgical Oncology Program at the Children's Hospital of Colorado, University of Colorado, Denver, Colorado, USA.
Ethan A. Smith, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA.
Jennifer Aldrink, Division of Pediatric Surgery, Department of Surgery, Nationwide Children's Hospital, The Ohio State University College of Medicine, Columbus, Ohio, USA.
Rodrigo L. Romao, Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.
Jeffrey S. Dome, Children National Hospital, George Washington University School of Medicine and Health Sciences, Washington, DC, USA.
Andrew M. Davidoff, St Jude Children's Research Hospital, Memphis, Tennessee, USA.
Amy L. Treece, Ann & Robert H. Lurie Children's Hospital, Chicago, Illinois, USA.
Lauren N. Parsons, Department of Pathology, Children's Hospital of Wisconsin, Milwaukee, Wisconsin, USA.
Elizabeth A. Mullen, Boston Children's Hospital and Dana Farber Cancer Institute, Boston, Massachusetts, USA.
Robert C. Shamberger, Boston Children's Hospital and Dana Farber Cancer Institute, Boston, Massachusetts, USA.
Arnold C. Paulino, MD Anderson Cancer Center, Houston, Texas, USA.
Andrea C. Lo, University of British Columbia, Vancouver, British Columbia, Canada.
James I. Geller, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA.
Peter F. Ehrlich, Section of Pediatric Surgery, CS Mott Children's Hospital, University of Michigan, Ann Arbor, Michigan, USA.

Document Type

Journal Article

Publication Date

7-8-2024

Journal

International journal of cancer

DOI

10.1002/ijc.35080

Keywords

bilateral Wilms tumor; events; long‐term outcomes; predisposition; relapse

Abstract

The objective of this study is to report the long-term timing and patterns of relapse for children enrolled in Children's Oncology Group AREN0534, a multicenter phase III clinical trial conducted from 2009 to 2015. Participants included children with bilateral Wilms tumor (BWT) or unilateral WT with genetic predisposition to develop BWT followed for up to 10 years. Smoothed hazard (risk) functions for event-free survival (EFS) were plotted so that the timing of events could be visualized, both overall and within pre-specified groups. Two hundred and twenty-two children (190 BWT and 32 unilateral WT with BWT predisposition) were followed for a median of 8.6 years. Fifty events were reported, of which 48 were relapse/progression. The overall 8-year EFS was 75% (95% confidence interval: 69%-83%). The highest risk for an EFS event was immediately after diagnosis with a declining rate over 2 years. A second peak of events was observed around 4 years after diagnosis, and a small number of events were reported until the end of the follow-up period. In subset analyses, later increases in risk were more commonly observed in patients with female sex, anaplastic histology, negative lymph nodes or margins, and favorable histology Wilms tumor patients with post-chemotherapy intermediate risk. Among relapses that occurred after 2 years, most were to the kidney. These patterns suggest that late events may be second primary tumors occurring more commonly in females, although more investigation is required. Clinicians may consider observation of patients with BWT beyond 4 years from diagnosis.

Department

Pediatrics

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