Wilms' tumor is a rare kidney cancer that primarily affects children.

Also known as
nephroblastoma, it's the most common malignant tumor of the kidneys in children. The
peak time of Wilms' tumor occurrence is around ages 3 to 4, and it occurs only rarely
after age 6.

Although Wilms' tumor can occur in both kidneys, it tends to affect just one kidney.
Wilms' tumor is believed to develop from immature kidney cells.

Improved imaging techniques help doctors to determine the extent of the cancer in
Wilms' tumor and to plan treatment. The outlook for most children with Wilms' tumor is
very good.

Symptoms

Wilms' tumor may also go undetected early on, because the tumors can grow large
without causing pain. However, even when they're large, most of these tumors are found
before they have a chance to spread (metastasize) to other parts of the body. Your child
may appear healthy or may have:

• Abdominal swelling
• An abdominal mass you can feel
• Fever
• Blood in the urine
• Reduced appetite
• High blood pressure
• Constipation
• Stomach pain
• Nausea

When to see a doctor

Take your child to the doctor if you detect an abdominal mass or if you notice blood in
the urine. There may be other signs and symptoms, such as fever, high blood pressure,
constipation, reduced appetite, abdominal pain and weight loss. These symptoms are
much more likely to be caused by something other than Wilms' tumor, but see your
child's doctor to evaluate them.

If your child has been treated for Wilms' tumor, report any new signs or symptoms to
your child's doctor. These problems may be a sign of cancer returning or of side effects of
treatment.

Causes

Although the exact cause remains unknown, it's believed that this cancer arises when
your child is still developing in the womb and some of the cells that are supposed to form
the kidneys fail to develop properly. Instead, they multiply in their primitive state,
becoming a tumor that often becomes evident around the time a child is 3 or 4 years old.

Some cancers, such as Wilms' tumor, occur when changes (mutations) arise in your
child's genes that control growth, allowing cells to multiply without restraint. In some
cases, cancer is caused by genetic defects passed from parent to child. For example, some
cases of Wilms' tumor are related to defects in one of two genes — either Wilms' tumor 1
(WT1) or Wilms' tumor 2 (WT2). Scientists believe that mutations in other chromosomes
also may play a role in Wilms' tumor.

In other cases, there is no known familial genetic defect. Instead, something happens in
the child's early development that somehow changes a gene and results in cancer.