Risk Factors for Wilms Tumors

Age

Wilms tumors are most common in young children, with the average age being about 3 to 4 years. They are less common in older children, and rare in adults.

Race/ethnicity

In the United States, the risk of Wilms tumor is slightly higher in Black children than in White children, and it’s lower among Asian American children. The reason for this is not known.

Sex

Wilms tumors are slightly more common in girls than in boys.

Family history of Wilms tumor

About 1% to 2% of children with Wilms tumors have one or more relatives with the same cancer. These children most likely inherit chromosomes with an abnormal or missing gene from a parent that increases their risk of developing Wilms tumor.

Children with a family history of Wilms tumors are slightly more likely to have tumors in both kidneys. Still, in most children only one kidney is affected.

Certain genetic syndromes/birth defects

There is a strong link between Wilms tumors and certain kinds of birth defects. About 1 child in 10 with a Wilms tumor also has one or more birth defects.

Most birth defects linked to Wilms tumors occur in syndromes. A syndrome is a group of symptoms, signs, malformations, or other abnormalities that occur together in the same person. Syndromes linked to Wilms tumor include:

WAGR syndrome

WAGR syndrome is linked with certain physical and mental issues (although not all children have all of them):

• Wilms tumor
• Aniridia (complete or partial lack of the iris [colored area] of the eyes)
• Genitourinary tract abnormalities (defects of the kidneys, urinary tract, penis, scrotum, clitoris, testicles, or ovaries)
• Range of developmental delays (including intellectual disabilities)

Children with this syndrome have about a 50% chance of developing a Wilms tumor. When they do develop Wilms tumors, it tends to happen at an earlier age, and often in both kidneys.

The cells in children with WAGR syndrome are missing part of chromosome 11, where the WT1 gene is normally found (see What Causes Wilms Tumors?).

Denys-Drash syndrome and Frasier syndrome

These rare syndromes have also been linked to changes (mutations) in the WT1 gene.

In Denys-Drash syndrome, the kidneys become diseased and stop working when the child is very young. Wilms tumors usually develop in the diseased kidneys. The reproductive organs don’t develop normally, and boys may be mistaken for girls. Because the risk of Wilms tumors is very high, doctors often advise removing the kidneys soon after this syndrome is diagnosed.

In Frasier syndrome the kidneys are also diseased, but they usually keep working into adolescence. As with Denys-Drash syndrome, the reproductive organs don’t develop normally. Children with Frasier syndrome are also at increased risk for Wilms tumors, although this risk is not as high as with Denys-Drash syndrome. They are at even higher risk for cancers in the reproductive organs.

Beckwith-Wiedemann syndrome

Children with this syndrome tend to be big for their age. They also have larger than normal internal organs and often have an enlarged tongue. They may have an oversized arm and/or leg on one side of the body (called hemihypertrophy), as well as other medical problems. They have about a 5% risk of Wilms tumors (or, less often, other cancers that develop during childhood).

This syndrome is caused by a defect in chromosome 11 that affects several genes.

Other syndromes

Less often, Wilms tumor has been linked to other syndromes, including:

• Perlman syndrome
• Fanconi anemia
• Sotos syndrome
• Simpson-Golabi-Behmel syndrome
• Bloom syndrome
• Li-Fraumeni syndrome
• Trisomy 18

Certain birth defects

Wilms tumor is also more common in children with certain birth defects (without known syndromes):

• Aniridia (complete or partial lack of the iris [colored area] of the eyes)
• Hemihypertrophy (an oversized arm and/or leg on one side of the body)
• Cryptorchidism (failure of the testicles to descend into the scrotum) in boys

Hypospadias (defect in boys where the urinary opening is on the underside of the penis)