Risks for Wilms tumour

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Some things can affect your risk, or chance, of developing cancer. Certain behaviours, substances or conditions can increase or decrease the risk. Most cancers are the result of many risks. Childhood cancers are rare, and there is less known about the risks. Most risks for childhood cancer are not modifiable, which means that you can't change them.

Risks for Wilms tumour include certain genetic conditions and a family history of the disease.

Most Wilms tumours are diagnosed in children younger than 5. Wilms tumours occur slightly more often in children of African ancestry and less often in children of Asian ancestry. They occur slightly more often in girls than boys.

Some Wilms tumours are caused by genetic changes children are born with and that are inherited from their parents. Others are caused by changes that happen in their cells after they are born, for reasons unrelated to their parents. Talk to your doctor about your child's risk for Wilms tumour.

The following can increase the risk for Wilms tumour. None of these risks can be changed. Until we learn more about these risks, there are no specific ways to lower the risk for Wilms tumour.

WAGR syndrome

Denys-Drash syndrome

Simpson-Golabi-Behmel syndrome

Frasier syndrome

Beckwith-Wiedemann syndrome

Perlman syndrome

Bloom syndrome

Li-Fraumeni syndrome

Aniridia

Family history

WAGR syndrome

WAGR syndrome is a rare genetic condition. It's caused by a change in a a tumour suppressor gene. Tumour suppressor genes help control cell growth and stop tumours from growing. When these genes don’t work properly, they no longer control cell growth and tumours may form.

WAGR stands for the 4 conditions that may be present in WAGR syndrome:

  • Wilms tumour
  • aniridia, which is when the coloured part of the eye (the iris) doesn’t form completely
  • genitourinary anomalies, which means the reproductive system and urinary system don’t form normally
  • intellectual disability

Most people who have WAGR syndrome have 2 or more of these conditions.

Denys-Drash syndrome

Denys-Drash syndrome is a genetic condition that causes kidney disease within the first few months of life. It also affects the sex organs so they don’t form properly.

Most children with Denys-Drash syndrome develop Wilms tumour in one or both kidneys. It's usually diagnosed around 2 years of age.

Simpson-Golabi-Behmel syndrome

Simpson-Golabi-Behmel syndrome is a genetic condition found mostly in boys. It affects many parts of the body. Babies with Simpson-Golabi-Behmel syndrome are much larger than normal at birth, and they grow and gain weight at an unusual rate.

Some children with Simpson-Golabi-Behmel syndrome will develop non-cancerous or cancerous tumours during childhood. The most common of these cancers is Wilms tumour.

Frasier syndrome

Frasier syndrome is a very rare genetic condition. In boys, Frasier syndrome affects both the kidneys and the genitals, so they don’t form properly. In girls, it usually only affects the kidneys.

Beckwith-Wiedemann syndrome

Beckwith-Wiedemann syndrome is an inherited condition that affects how different parts of the body grow. It's caused by changes in a certain chromosome– the part of a cell that contains DNA. Signs of Beckwith-Wiedemann syndrome include being heavy when born, being talller than average during childhood, a large tongue and ears, a defect in the abdominal wall and low blood sugar in newborns.

Perlman syndrome

Perlman syndrome is a very rare genetic condition. It's very similar to Beckwith-Wiedemann syndrome. It can occur when there is too much fluid in the mother's uterus during pregnancy. As a result, the baby may be born larger and heavier than normal, with abnormal facial features.

Babies and children with Perlman syndrome have a higher risk of developing Wilms tumour.

Bloom syndrome

Bloom syndrome is an inherited condition caused by mutations in a certain chromosome. Signs include shorter than average height, a high-pitched voice and a characteristic facial appearance.

Li-Fraumeni syndrome

Li-Fraumeni syndrome is an inherited condition that is associated with a higher risk for certain types of cancers, including Wilms tumour.

Aniridia

Aniridia is a rare birth defect in which the coloured part of the eye (the iris) doesn’t form completely. The iris controls the amount of light entering the eye. Aniridia can cause blindness.

Aniridia is part of WAGR syndrome, but it can also increase the risk of Wilms tumour on its own.

Family history

A small number of Wilms tumours develop in children who have family members with the disease.

Possible risks

The following have been linked with Wilms tumour, but more research is needed to know for sure that they are risks.

  • high birth weight (more than 4 kilograms at birth)
  • hypospadias–a birth defect in boys when the urinary (urethral) opening is on the underside of the penis rather than at the tip

Expert review and references

  • Canadian Cancer Society | Société canadienne du cancer
  • American Cancer Society. Risk Factors for Wilms Tumors. 2025. https://www.cancer.org/.
  • Chu A, Heck JE, Ribeiro KB, et al. Wilms' tumour: a systematic review of risk factors and meta-analysis. Paediatric and Perinatal Epidemiology. England: Blackwell Scientific Publications; 2010.
  • Cancer Research UK. What is Wilms Tumour?. 2023. https://www.cancerresearchuk.org/.
  • Fear NT, Vincent TJ, King JC, et al. Wilms tumour and paternal occupation: an analysis of data from the National Registry of Childhood Tumours. Pediatric Blood & Cancer. Wiley Online; 2010.
  • Fernandez CV, Geller JI, Ehrlich PF, van den Heuvel-Eibrink, MM, et al. Renal tumors. Blaney SM, Adamson PC, Helman LJ (eds.). Pizzo and Pollack's Pediatric Oncology. 8th ed. Wolters Kluwer; 2021: Kindle version, [chapter 24] https://read.amazon.ca/?asin=B08DVWZNVP&language=en-CA.
  • National Library of Medicine. Medline Plus Genetics Beckwith-Wiedemann Syndrome. 2021. https://medlineplus.gov/genetics/.
  • National Library of Medicine. MedlinePlus: Genetics Bloom Syndrome. 2015. https://medlineplus.gov/genetics/.
  • National Library of Medicine. MedlinePlus Genetics Denys-Drash Syndrome. 2013. https://medlineplus.gov/genetics/.
  • National Library of Medicine. Medline Plus Genetics Frasier Syndrome. 2013. https://medlineplus.gov/genetics/.
  • National LIbrary of Medicine. Medline Plus Genetics Li-Fraumeni Syndrome. 2020. https://medlineplus.gov/genetics/.
  • National Library of Medicine. MedlinePlus Genetics: WAGR Syndrome. 2023. https://medlineplus.gov/genetics/.
  • Rangel M, Cypriano M, de Martino Lee ML, et al. Leukemia, non-Hodgkin's lymphoma, and Wilms tumor in childhood: the role of birth weight. European Journal of Pediatrics. Springer; 2010.
  • Roman E, Lightfoot T, Picton S and Kinsey S. Childhood Cancers. Thun MJ (ed.). Schottenfeld and Fraumeni Cancer Epidemiology and Prevention. 4th ed. New York, NY: Oxford University Press; 2018: 59:1119-1153.
  • Schuz J, Schmidt LS, Kogner P, et al. Birth characteristics and Wilms tumors in children in the Nordic countries: a register-based case-control study. International Journal of Cancer. Hoboken, NJ: Wiley-Liss, Inc; 2011.

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