Highlights
- •Bilateral Wilms tumour (BWT) is rare, with reported worse outcomes than unilateral disease. Canadian outcome data has never been published.
- •This is the first epidemiologic study of outcomes for Canadian BWT patients. Overall survival improved when patients were treated using a specific therapeutic protocol (AREN0534), and late events were rare.
Abstract
Background
Bilateral Wilms tumour (BWT) is a rare entity. The goal of this study is to report
outcomes (overall and event-free survival, OS/EFS) of BWT in a large cohort representative
of the Canadian population since 2000. We focused on the occurrence of late events
(relapse or death beyond 18 months), as well as outcomes of patients treated following
the only protocol specifically designed for BWT to date, AREN0534, compared to patients
treated following other therapeutic schemes.
Methods
Data was obtained for patients diagnosed with BWT between 2001 – 2018 from the Cancer
in Young People in Canada (CYP-C) database. Demographics, treatment protocols, and
dates for events were collected. Specifically, we examined outcomes of patients treated
according to the Children’s Oncology Group (COG) protocol AREN0534 since 2009. Survival
analysis was performed.
Results
57/816 (7%) of patients with Wilms tumour had BWT during the study period. Median
age at diagnosis was 2.74 years (IQR 1.37-4.48) and 35 (64%) were female; 8/57 (15%)
had metastatic disease. After a median follow-up of 4.8 years (IQR 2.8-5.7 years,
range 0.2-18 years), OS and EFS were 86% (CI 73-93%) and 80% (CI 66-89%), respectively.
Less than 5 events were recorded after 18 months from diagnosis. Since 2009, patients
treated according to the AREN0534 protocol had a statistically significant higher
OS compared to patients treated with other protocols.
Conclusions
In this large Canadian cohort of patients with BWT, OS and EFS compared favorably
to the published literature. Late events were rare. Patients treated according to
a disease-specific protocol (AREN0534) had improved overall survival.
Level of Evidence
Level IV
Keywords
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Article info
Publication history
Accepted:
January 2,
2023
Received:
December 16,
2022
Publication stage
In Press Accepted ManuscriptFootnotes
☆This is an original article.
☆☆The authors have no competing interests to declare.
☆☆☆This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors
Identification
Copyright
© 2023 Elsevier Inc. All rights reserved.
