Globally, the age‐standardised incidence rate (ASR) of Wilms' tumour is 7.5 per million.[1]Nakata K, Colombet M, Stiller CA, et al. Incidence of childhood renal tumours: an international population-based study. Int J Cancer. 2020 Dec 15;147(12):3313-27.
https://onlinelibrary.wiley.com/doi/10.1002/ijc.33147
http://www.ncbi.nlm.nih.gov/pubmed/32902866?tool=bestpractice.com
South Asia has the lowest ASR of 3.6 per million; North America and Western Europe have the highest ASRs of 8.5 per million and 9.6 per million, respectively.[1]Nakata K, Colombet M, Stiller CA, et al. Incidence of childhood renal tumours: an international population-based study. Int J Cancer. 2020 Dec 15;147(12):3313-27.
https://onlinelibrary.wiley.com/doi/10.1002/ijc.33147
http://www.ncbi.nlm.nih.gov/pubmed/32902866?tool=bestpractice.com
In the US, approximately 7% of new cancers diagnosed each year in children aged <15 years are renal cancers; approximately 80% of these are Wilms' tumour.[15]Geller JI, Hong AL, Vallance KL, et al. Children's Oncology Group's 2023 blueprint for research: renal tumors. Pediatr Blood Cancer. 2023 Sep;70 Suppl 6(suppl 6):e30586.
https://onlinelibrary.wiley.com/doi/10.1002/pbc.30586
http://www.ncbi.nlm.nih.gov/pubmed/37477907?tool=bestpractice.com
The incidence of Wilms' tumour in the US is highest in non-Hispanic American Indian/Alaska Native and non-Hispanic black children (8.6 and 8.1 per million, respectively, age-adjusted incidence rates 2017-2021) and lowest in Non-Hispanic Asian/Pacific Islander children (3.7 per million).[16]National Cancer Institute. Statistics for cancers in children, adolescents, and young adults. Oct 2024 [internet publication].
https://nccrexplorer.ccdi.cancer.gov
Incidence rates are higher in females than for males (6.4 vs. 5.5 per million, 2017-2021 data). The incidence of Wilms' tumour has remained relatively stable over time.[2]Espinoza AF, Onwuka E, Siegel DA, et al. Incidence and survival of children and adolescents with Wilms tumor, United States, 2001-2020. Cancer Med. 2025 Feb;14(3):e70598.
https://onlinelibrary.wiley.com/doi/10.1002/cam4.70598
http://www.ncbi.nlm.nih.gov/pubmed/39928531?tool=bestpractice.com
[16]National Cancer Institute. Statistics for cancers in children, adolescents, and young adults. Oct 2024 [internet publication].
https://nccrexplorer.ccdi.cancer.gov
Wilms' tumour most commonly occurs in the first 5 years of life (median age at diagnosis is 4 years).[2]Espinoza AF, Onwuka E, Siegel DA, et al. Incidence and survival of children and adolescents with Wilms tumor, United States, 2001-2020. Cancer Med. 2025 Feb;14(3):e70598.
https://onlinelibrary.wiley.com/doi/10.1002/cam4.70598
http://www.ncbi.nlm.nih.gov/pubmed/39928531?tool=bestpractice.com
[3]Lovvorn HN 3rd, Renfro LA, Benedetti DJ, et al. Race and ethnic group enrollment and outcomes for Wilms tumor: analysis of the current era children's oncology group study, AREN03B2. J Am Coll Surg. 2024 Apr 1;238(4):733-49.
http://www.ncbi.nlm.nih.gov/pubmed/38251681?tool=bestpractice.com
Bilateral disease tends to present at a slightly younger age (median age at diagnosis <2 years) than unilateral disease and is more prevalent in patients with a Wilms' tumour predisposition syndrome.[1]Nakata K, Colombet M, Stiller CA, et al. Incidence of childhood renal tumours: an international population-based study. Int J Cancer. 2020 Dec 15;147(12):3313-27.
https://onlinelibrary.wiley.com/doi/10.1002/ijc.33147
http://www.ncbi.nlm.nih.gov/pubmed/32902866?tool=bestpractice.com
[6]Charlton J, Irtan S, Bergeron C, et al. Bilateral Wilms tumour: a review of clinical and molecular features. Expert Rev Mol Med. 2017 Jul 18;19:e8.
https://www.cambridge.org/core/journals/expert-reviews-in-molecular-medicine/article/bilateral-wilms-tumour-a-review-of-clinical-and-molecular-features/B4C8FBEE8C2C189D7D739B9BD64F1AA8
http://www.ncbi.nlm.nih.gov/pubmed/28716159?tool=bestpractice.com
Approximately 21% of patients have metastatic disease at presentation; lungs are the most common site for metastases.[3]Lovvorn HN 3rd, Renfro LA, Benedetti DJ, et al. Race and ethnic group enrollment and outcomes for Wilms tumor: analysis of the current era children's oncology group study, AREN03B2. J Am Coll Surg. 2024 Apr 1;238(4):733-49.
http://www.ncbi.nlm.nih.gov/pubmed/38251681?tool=bestpractice.com