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Outcomes according to treatment using an established protocol in patients with bilateral Wilms’ tumour: A national Canadian population-based study

      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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      References

        • Davidoff A.M.
        • Interiano R.B.
        • Wynn L.
        • et al.
        Overall Survival and Renal Function of Patients With Synchronous Bilateral Wilms Tumor Undergoing Surgery at a Single Institution.
        Ann Surg. 2015; 262: 570-576https://doi.org/10.1097/SLA.0000000000001451
      1. Statistics Canada. Childhood kidney and renal pelvis cancer statistics. Canadian Cancer Society, https://cancer.ca/en/cancer-information/cancer-types/wilms-tumour/statistics#:∼:text=Incidence%20is%20the%20total%20number,kidney%20and%20renal%20pelvis%20cancer; 2022 [accessed 21 July 2022].

        • Spreafico F.
        • Fernandez C.V.
        • Brok J.
        • et al.
        Wilms tumour.
        Nat Rev Dis Primer. 2021; 7: 75https://doi.org/10.1038/s41572-021-00308-8
        • Ehrlich P.F.
        • Chi Y.-Y.
        • Chintagumpala M.M.
        • et al.
        Results of the First Prospective Multi-institutional Treatment Study in Children With Bilateral Wilms Tumor (AREN0534) A Report From the Children’s Oncology Group.
        Ann Surg. 2018; 267: e64https://doi.org/10.1097/SLA.0000000000002696
        • Hamilton T.E.
        • Ritchey M.L.
        • Haase G.M.
        • et al.
        The Management of Synchronous Bilateral Wilms Tumor: A Report from the National Wilms Tumor Study Group.
        Ann Surg. 2011; 253: 1004-1010https://doi.org/10.1097/SLA.0b013e31821266a0
        • Dome J.S.
        • Cotton C.A.
        • Perlman E.J.
        • et al.
        Treatment of Anaplastic Histology Wilms’ Tumor: Results From the Fifth National Wilms’ Tumor Study.
        J Clin Oncol. 2006; 24: 2352-2358https://doi.org/10.1200/JCO.2005.04.7852
        • Brok J.
        • Lopez-Yurda M.
        • Tinteren H.V.
        • et al.
        Relapse of Wilms’ tumour and detection methods: a retrospective analysis of the 2001 Renal Tumour Study Group–International Society of Paediatric Oncology Wilms’ tumour protocol database.
        Lancet Oncol. 2018; 19: 1072-1081https://doi.org/10.1016/S1470-2045(18)30293-6
        • Charlton J.
        • Irtan S.
        • Bergeron C.
        • et al.
        Bilateral Wilms tumour: a review of clinical and molecular features.
        Expert Rev Mol Med. 2017; 19: e8https://doi.org/10.1017/erm.2017.8
        • Davidoff A.M.
        Wilms Tumor.
        Adv Pediatr. 2012; 59: 247-267https://doi.org/10.1016/j.yapd.2012.04.001
        • Han Q.
        • Li K.
        • Dong K.
        • et al.
        Clinical features, treatment, and outcomes of bilateral Wilms’ tumor: A systematic review and meta-analysis.
        J Pediatr Surg. 2018; 53: 2465-2469https://doi.org/10.1016/j.jpedsurg.2018.08.022
        • Doganis D.
        • Zborovskaya A.
        • Trojanowski M.
        • et al.
        Wilms tumour event-free and overall survival in Southern and Eastern Europe: Pooled analyses of clinical data from four childhood cancer registries (1999–2017).
        Eur J Cancer. 2019; 115: 37-46https://doi.org/10.1016/j.ejca.2019.04.008
        • Neu M.A.
        • Russo A.
        • Wingerter A.
        • et al.
        Prospective analysis of long-term renal function in survivors of childhood Wilms tumor.
        Pediatr Nephrol. 2017; 32: 1915-1925https://doi.org/10.1007/s00467-017-3673-9
        • Aronson D.C.
        • Slaar A.
        • Heinen R.C.
        • et al.
        Long-term outcome of bilateral Wilms tumors (BWT).
        Pediatr Blood Cancer. 2011; 56: 1110-1113https://doi.org/10.1002/pbc.22881
      2. Combination Chemotherapy and Surgery in Treating Young Patients With Wilms Tumor. Children’s Oncology Group, https://clinicaltrials.gov/ct2/show/NCT00945009; 2009 [accessed July 21, 2022].

        • Johnston D.L.
        • Keene D.
        • Bartels U.
        • et al.
        Patterns of enrollment of infants with central nervous system tumours on cooperative group studies: a report from the Canadian Pediatric Brain Tumour Consortium.
        J Neurooncol. 2010; 99: 243-249https://doi.org/10.1007/s11060-010-0123-y
        • Peppercorn J.M.
        • Weeks J.C.
        • Cook E.F.
        • et al.
        Comparison of outcomes in cancer patients treated within and outside clinical trials: conceptual framework and structured review.
        The Lancet. 2004; 363: 263-270https://doi.org/10.1016/S0140-6736(03)15383-4
        • Public Health Agency of Canada
        Release notice - Cancer in Young People in Canada: a report from the Enhanced Childhood Cancer Surveillance System.
        Health Promot Chronic Dis Prev Can. 2017; 37 (393): 393https://doi.org/10.24095/hpcdp.37.11.04