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Wilms Tumor and Other Childhood Kidney Tumors Treatment (PDQ®): Treatment - Patient Information [NCI] - General Information About Wilms Tumor and Other Childhood Kidney Tumors


Having certain genetic syndromes or other conditions can increase the risk of Wilms tumor.

Anything that increases the risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk to your child's doctor if you think your child may be at risk.

Wilms tumor may be part of a genetic syndrome that affects growth or development. A genetic syndrome is a set of signs and symptoms or conditions that occur together and is caused by certain changes in the genes. Certain conditions can also increase a child's risk of developing Wilms tumor. These and other genetic syndromes and conditions have been linked to Wilms tumor:

  • WAGR syndrome (Wilms tumor, aniridia, abnormal genitourinary system, and mental retardation).
  • Beckwith-Wiedemann syndrome (abnormally large growth of one side of the body or a body part, large tongue, umbilical hernia at birth, and abnormal genitourinary system).
  • Denys-Drash syndrome (abnormal genitourinary system).
  • Frasier syndrome (abnormal genitourinary system).
  • Isolated hemihypertrophy (abnormally large growth of one side of the body or a body part).
  • Urinary tract problems such as cryptorchidism or hypospadias.
  • Aniridia (the iris, the colored part of the eye, is missing).
  • A family history of Wilms tumor.

Tests are used to screen for Wilms tumor.

Screening tests are done in children with an increased risk of Wilms tumor. These tests may help find cancer early and decrease the chance of dying from cancer.

In general, children with an increased risk of Wilms tumor should be screened for Wilms tumor every three months until they are at least 8 years old. An ultrasound test of the abdomen is usually used for screening. Small Wilms tumors may be found early and removed.

Children with Beckwith-Wiedemann syndrome or hemihypertrophy are also screened for liver and adrenal tumors that are linked to these genetic syndromes. A test to check the alpha-fetoprotein (AFP) level in the blood and an ultrasound of the abdomen are done until the child is 4 years old. An ultrasound of the kidneys is done after the child is 4 years old.

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