There are no specific prevention strategies for Wilms' tumour; however, tumour surveillance strategies should be employed to ensure early detection in children with genetic syndromes that increase the risk of Wilms' tumour.[4]Spreafico F, Fernandez CV, Brok J, et al. Wilms tumour. Nat Rev Dis Primers. 2021 Oct 14;7(1):75.
https://www.nature.com/articles/s41572-021-00308-8
http://www.ncbi.nlm.nih.gov/pubmed/34650095?tool=bestpractice.com
[37]Kalish JM, Becktell KD, Bougeard G, et al. Update on surveillance for Wilms tumor and hepatoblastoma in Beckwith-Wiedemann syndrome and other predisposition Ssyndrome. Clin Cancer Res. 2024 Dec 2;30(23):5260-9.
https://aacrjournals.org/clincancerres/article/30/23/5260/750189/Update-on-Surveillance-for-Wilms-Tumor-and
http://www.ncbi.nlm.nih.gov/pubmed/39320341?tool=bestpractice.com
Renal ultrasound every 3 months from birth until aged 7 years is recommended for children with a Wilms' tumour predisposition syndrome.[37]Kalish JM, Becktell KD, Bougeard G, et al. Update on surveillance for Wilms tumor and hepatoblastoma in Beckwith-Wiedemann syndrome and other predisposition Ssyndrome. Clin Cancer Res. 2024 Dec 2;30(23):5260-9.
https://aacrjournals.org/clincancerres/article/30/23/5260/750189/Update-on-Surveillance-for-Wilms-Tumor-and
http://www.ncbi.nlm.nih.gov/pubmed/39320341?tool=bestpractice.com
[38]Brzezinski JJ, Becktell KD, Bougeard G, et al. Update on surveillance guidelines in emerging Wilms tumor predisposition syndromes. Clin Cancer Res. 2025 Jan 6;31(1):18-24.
https://aacrjournals.org/clincancerres/article/31/1/18/750711/Update-on-Surveillance-Guidelines-in-Emerging
http://www.ncbi.nlm.nih.gov/pubmed/39466169?tool=bestpractice.com
[39]Hol JA, Jewell R, Chowdhury T, et al. Wilms tumour surveillance in at-risk children: literature review and recommendations from the SIOP-Europe Host Genome Working Group and SIOP Renal Tumour Study Group. Eur J Cancer. 2021 Aug;153:51-63.
https://www.ejcancer.com/article/S0959-8049(21)00322-1/fulltext
http://www.ncbi.nlm.nih.gov/pubmed/34134020?tool=bestpractice.com
Genetic and antenatal counselling should be offered to parents with a family history of Wilms' tumour.[40]Schienda J, Stopfer J. Cancer genetic counseling-current practice and future challenges. Cold Spring Harb Perspect Med. 2020 Jun 1;10(6):a036541.
https://perspectivesinmedicine.cshlp.org/content/10/6/a036541.long
http://www.ncbi.nlm.nih.gov/pubmed/31548230?tool=bestpractice.com
[41]Turner JT, Brzezinski J, Dome JS. Wilms tumor predisposition. 2003 Dec 19 (updated 2022 Mar 24). In: Adam MP, Mirzaa GM, Pagon RA, et al, eds. GeneReviews [internet]. Seattle, WA: University of Washington, Seattle; 1993-2023.
https://www.ncbi.nlm.nih.gov/books/NBK1294
http://www.ncbi.nlm.nih.gov/pubmed/20301471?tool=bestpractice.com
Children at very high risk
Children with a germline WT1 mutation (such as those with Denys-Drash syndrome) are at very high risk of developing Wilms' tumour and have an increased risk of end-stage kidney disease at a young age.[42]Lopez-Gonzalez M, Ariceta G. WT1-related disorders: more than Denys-Drash syndrome. Pediatr Nephrol. 2024 Sep;39(9):2601-9.
http://www.ncbi.nlm.nih.gov/pubmed/38326647?tool=bestpractice.com
Risk of development of renal insufficiency and Wilms' tumour varies according to the type and location of WT1 mutation.[43]Lipska BS, Ranchin B, Iatropoulos P, et al. Genotype-phenotype associations in WT1 glomerulopathy. Kidney Int. 2014 May;85(5):1169-78.
https://www.kidney-international.org/article/S0085-2538(15)56331-X/fulltext
http://www.ncbi.nlm.nih.gov/pubmed/24402088?tool=bestpractice.com
[44]Lehnhardt A, Karnatz C, Ahlenstiel-Grunow T, et al. Clinical and molecular characterization of patients with heterozygous mutations in wilms tumor suppressor gene 1. Clin J Am Soc Nephrol. 2015 May 7;10(5):825-31.
http://www.ncbi.nlm.nih.gov/pubmed/25818337?tool=bestpractice.com
In these patients, special consideration should be given to balancing the risks of renal salvage, renal replacement therapy, and anti-cancer therapy.