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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 birth defects 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 symptoms or conditions that occur together and is usually caused by abnormal genes. Certain birth defects can also increase a child's risk for developing Wilms tumor. The following genetic syndromes and birth defects have been linked to Wilms tumor:

  • WAGR syndrome (Wilms tumor, aniridia, abnormal genitourinary system, and mental retardation).
  • Beckwith-Wiedemann syndrome.
  • Idiopathic hemihypertrophy (abnormally large growth of one side of the body or a body part).
  • Denys-Drash syndrome.
  • Cryptorchidism.
  • Hypospadias.

Children with these genetic syndromes and birth defects should be screened for Wilms tumor every three months until at least age 8. An ultrasound test of the abdomen may be used for screening. 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 level in the blood and an ultrasound of the abdomen are done until age 4 years. An ultrasound of the kidneys is done after age 4 years.

Some children develop Wilms tumor in both kidneys. These often appear when Wilms tumor is first diagnosed, but a Wilms tumor may also occur in the second kidney after the child is successfully treated for Wilms tumor in one kidney. A second tumor is much more likely to develop in the other kidney when a child's first Wilms tumor is diagnosed before age 12 months or when embryonic cells remain in the kidney.

Children at risk for developing a second Wilms tumor in the other kidney should be screened for Wilms tumor every three months for two to six years. An ultrasound test of the abdomen may be used for screening.

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