Wilms' tumor is a rare kidney cancer that primarily affects children. Also known as nephroblastoma, it's the most common cancer of the kidneys in children. Wilms' tumor most often affects children ages 3 to 4 and becomes much less common after age 5.

Wilms' tumor most often occurs in just one kidney, though it can sometimes be found in both kidneys at the same time.

Symptoms

Signs and symptoms of Wilms' tumor vary widely, and some children don't show any obvious signs. But most children with Wilms' tumor experience one or more of these signs and symptoms:

• An abdominal mass you can feel
• Abdominal swelling
• Abdominal pain

Other signs and symptoms may include:

• Fever
• Blood in the urine
• Nausea or vomiting or both
• Constipation
• Loss of appetite
• Shortness of breath
• High blood pressure

Causes

It's not clear what causes Wilms' tumor, but in rare cases, heredity may play a role.

Cancer begins when cells develop errors in their DNA. The errors allow the cells to grow and divide uncontrollably and to go on living when other cells would die. The accumulating cells form a tumor. In Wilms' tumor, this process occurs in the kidney cells.

In rare cases, the errors in DNA that lead to Wilms' tumor are passed from a parent to the child. In most cases, there is no known connection between parents and children that may lead to cancer.

Diagnosis

To diagnose Wilms' tumor, your child's doctor may recommend:

• A physical exam
• Blood and urine tests
• Imaging tests

Staging

In the United States, guidelines developed through the National Wilms Tumor Study of the Children's Oncology Group include these five stages:

Stage I. The cancer is found only in one kidney, is completely contained within the kidney, and can be completely removed with surgery.

Stage II. The cancer has spread to the tissues and structures beyond the affected kidney, such as nearby fat or blood vessels, but it can still be completely removed by surgery.

Stage III. The cancer has spread beyond the kidney area to nearby lymph nodes or other structures within the abdomen, the tumor may spill within the abdomen before or during surgery, or it may not be completely removed by surgery.

Stage IV. The cancer has spread outside the kidney to distant structures, such as the lungs, liver, bones or brain.

Stage V. Cancer cells are found in both kidneys (bilateral tumors).

Treatment

Treatment for Wilms' tumor usually involves surgery and chemotherapy, and sometimes radiation therapy. Treatments may vary by the stage of the cancer. Because this type of cancer is rare, your child's doctor may recommend that you seek treatment at a children's cancer center that has experience treating this type of cancer.

Surgery to remove all or part of a kidney

• Chemotherapy
• Radiation therapy
• Clinical trials

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