Wilms Tumor - Childhood: Risk Factors

Approved by the Cancer.Net Editorial Board, 01/2022

ON THIS PAGE: You will find out more about the factors that increase the chance of developing a Wilms tumor. Use the menu to see other pages.

A risk factor is anything that increases a person’s chance of developing a tumor. Although risk factors often influence the development of a Wilms tumor, most do not directly cause it. Some children with several risk factors never develop a tumor, while others with no known risk factors do.

Most often, the risk of a Wilms tumor is not inherited, but there can be genetic reasons for the tumor’s development.

  • Genetic changes. Children may have a mutated (changed), damaged, or missing gene. This change can also cause other birth defects. About 15% of children with a Wilms tumor were born with other health problems.

  • WAGR syndrome. WAGR syndrome is a condition that causes a number of birth defects. Children with this syndrome have a 33% chance of developing a Wilms tumor. WAGR stands for:

    • Wilms tumor

    • Aniridia (no iris in the eye)

    • Genitourinary abnormalities, which are changes to the reproductive and urinary organs, or gonadoblastoma, which is a rare tumor of the reproductive organs

    • Intellectual disability

  • Beckwith-Wiedemann syndrome. This condition causes enlarged internal organs and limbs. Children with this syndrome have a higher risk of developing a Wilms tumor, kidney cysts, and tumors of the liver (hepatoblastoma), pancreas, and adrenal glands.

  • Denys-Drash syndrome. This is a rare condition in which male genital organs do not develop correctly and there is an increased risk of developing a Wilms tumor.

  • Family history. A small number of children with a Wilms tumor have a relative in their family with the disease.

The next section in this guide is Symptoms and Signs. It explains what changes or medical problems a Wilms tumor can cause. Use the menu to choose a different section to read in this guide.