Wilms Tumor

(Nephroblastoma; Kidney Tumor)

Definition

Wilms tumor is a type of kidney cancer. It’s mainly found in children. Most of the time, it’s found in one kidney, not both.

The Kidneys

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Causes    TOP

Before birth, some of kidney cells don’t mature like they’re supposed to. After the baby is born, clusters of these cells are still there. If they do not mature within 3 to 4 years, they may grow without control or order. A large mass of these cells may grow into a Wilms tumor.

Risk Factors    TOP

The chances of Wilms tumor are higher for:

  • Getting a certain gene from a parent that isn’t normal
  • Having someone in the family with same problems
  • Certain syndromes such as:
    • WAGR—a syndrome that includes:
      • W ilms tumor
      • A niridia—The colored part of the eye may be partly or fully missing.
      • G enitourinary abnormalities
      • R etardation
    • Beckwith-Wiedemann syndrome—Larger than normal tongue and organs. One arm or leg may be larger than the other.
    • Perlman syndrome—Large size before birth. May also have Wilms tumor. These may involve other organs and intellectual problems.
    • Denys-Drash syndrome—Male genitals are not present or completely formed.
    • Hemihypertrophy—One side of the body larger is than the other.
    • Other defects on chromosome 11.

Many children with Wilms tumors don’t have these problems. The cause of their kidney cells not maturing is unknown.

Symptoms    TOP

Wilms tumor may be first felt as a large mass in the belly. Other problems may include:

  • Stomach pain
  • Fever
  • Blood in the urine
  • Loss of hunger

Wilms tumors may grow larger without causing pain or other problems.

Diagnosis    TOP

You will be asked about your child’s symptoms and health history. During a physical exam, the doctor will look for signs of the syndromes listed above. These may point to a Wilms tumor.

The exam and test results will help find out the stage of cancer. Staging helps with planning. Wilms tumor is staged from 1-4. Stage 1 is a very localized cancer. Stage 4 is a spread to other parts of the body.

Treatment    TOP

Wilms tumor can be cured in most children. The plan depends on the stage of cancer. Many times, different methods are used. These may include:

Surgery

Surgery is done to remove as much cancer as possible. Nearby lymph nodes or other sites with cancer will also be removed. A nephrectomy may be:

  • Partial—removal of the cancerous part of the kidney to treat smaller tumors that have not spread
  • Radical—removal of the entire kidney and other nearby structures

Chemotherapy

Chemotherapy is the use of drugs to kill cancer cells. It may given by mouth, shots, or IV. The drugs enter the bloodstream and travel throughout the body.

Radiation Therapy    TOP

This is the use of radiation to kill cancer cells and shrink tumors after surgery. External radiation therapy is aimed at a tumor from a source outside the body.

Prevention    TOP

If you have Wilms tumor in your family, talk to your doctor about genetic testing before you start your own family. If your child is at high risk, talk to their doctor about screening. This may help find tumors early.

RESOURCES:

American Cancer Society
https://www.cancer.org
National Cancer Institute
https://www.cancer.gov

CANADIAN RESOURCES:

Childhood Cancer Canada
https://www.childhoodcancer.ca
Sick Kids—The Hospital for Sick Children
http://www.sickkids.ca

References:

Driskoll K, Isakoff M, Ferrer F. Update on pediatric genitourinary oncology. Curr Opin Urol. 2007;17(4):281-286.
Wilms tumor. EBSCO DynaMed Plus website. Available at: http://www.dynamed.com/topics/dmp~AN~T114137/Wilms-tumor. Updated July 26, 2017. Accessed July 31, 2018.
Wilms tumor. Kids Health—Nemours Foundation website. Available at: https://kidshealth.org/en/parents/wilms.html. Updated August 2016. Accessed July 31, 2018.
Wilms tumor. Merck Manual Professional Version website. Available at: https://www.merckmanuals.com/professional/pediatrics/pediatric-cancers/wilms-tumor. Updated August 2015. Accessed July 31, 2018.
Last reviewed June 2018 by EBSCO Medical Review Board Mohei Abouzied, MD, FACP
Last Updated: 7/31/2018

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