Health Information

Wilms Tumor

What is Wilms tumor?

Wilms tumor, also called nephroblastoma, is a malignant (cancerous) tumor originating in the cells of the kidney. It is the most common type of renal (kidney) cancer and accounts for about 6 percent of all childhood cancers.

Approximately 500 children in the US are diagnosed with Wilms tumor each year.

The disease can occur at any age between infancy and 15 years, but in most cases, the tumor is detected by the age of 3. It is slightly more common among girls and African-Americans.

The tumor can be very large and it may spread (metastasize) to other body tissues. The most common site for Wilms tumor to metastasize is the lungs. Lesions may also occur, however, in the liver, the other kidney, brain, and/or bones. In approximately 5 percent of children with Wilms tumor, both kidneys are involved.

What causes Wilms tumor?

It is uncommon for Wilms tumor to run in families. Less than 2 percent of cases will have an affected relative. Most cases of Wilms tumor are considered sporadic (occur by chance) and are the result of genetic mutations that affect cell growth in the kidney. These mutations generally arise after birth, but, in some cases, children are born with a genetic alteration that predisposes them to cancer.

A small percentage of patients with Wilms tumor have one of three genetic syndromes, including the following:

What are the symptoms of Wilms tumor?

The following are the most common symptoms of Wilms tumor. However, each child may experience symptoms differently. Symptoms may include:

If a tumor is suspected in the child's abdomen, it is important not to apply pressure to this area. Careful bathing and handling of the child is necessary before and during any tumor evaluation. Rupture of the tumor may lead to cancer cells spreading to other tissues in the body.

These symptoms of Wilms tumor may resemble other medical conditions or serious illnesses. Always consult your child's physician for a diagnosis.

How is Wilms tumor diagnosed?

In addition to a complete medical history and physical examination, diagnostic procedures may include, but are not limited to, the following:

Treatment for Wilms tumor:

Specific treatment for Wilms tumor will be determined by your child's physician based on:

Treatment may include (alone or in combination):

Long-term outlook for Wilms tumor:

The factors for determining the prognosis and long-term survival of children with Wilms tumor include the following:

As with any cancer, prognosis and long-term survival can vary greatly from child to child.

Prompt medical attention and aggressive therapy are important for the best possible prognosis. Continued follow-up care is essential for the child diagnosed with Wilms tumor. Side effects of chemotherapy and radiation, as well as second malignancies, can occur in survivors of cancer. New methods are continually being discovered to improve treatment and to decrease side effects.

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The WT1-related Wilms tumor (WT) syndromes are a group of hereditary disorders caused by alterations in a gene known as WT1. Learn more »