Faculty Collaboration Database

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. Int J Cancer 2024 Nov 15;155(10):1824-1831

Date

07/08/2024

Pubmed ID

38973574

Pubmed Central ID

PMC11570340

DOI

10.1002/ijc.35080

Scopus ID

2-s2.0-85197731115 (requires institutional sign-in at Scopus site)   10 Citations

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.

Author List

Murphy AJ, Brzezinski J, Renfro LA, Tornwall B, Malek MM, Benedetti DJ, Cost NG, Smith EA, Aldrink J, Romao RLP, Dome JS, Davidoff AM, Treece AL, Parsons LN, Mullen EA, Shamberger RC, Paulino AC, Lo AC, Geller JI, Ehrlich PF

Author

Lauren Parsons MD Associate Professor in the Pathology and Laboratory Medicine department at Medical College of Wisconsin

MESH terms used to index this publication - Major topics in bold

Child
Child, Preschool
Female
Follow-Up Studies
Genetic Predisposition to Disease
Humans
Infant
Kidney Neoplasms
Male
Neoplasm Recurrence, Local
Wilms Tumor