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Wilms' Tumor (WT)
Wednesday, 02 July 2003
Monday, 05 September 2005
Nephroblastoma, Wilm's Tumor

What

Wilms’ tumor is the most common form of childhood kidney cancer. The kidneys filter the blood, assist in retaining important proteins and other materials while at the same time removing waste through the production of urine. Children who develop Wilms’ tumor can have problems with urination but most commonly present with an enlarging abdominal mass.

Who

Nearly 75% of Wilms’ tumors are found in children less than 5 years of age. The average age of occurrence is 2-3 years old with a very low incidence in children greater than 10 years old. The tumor occurs in 0.8 out of 100,000 persons, with 500 new cases diagnosed each year in the United States. Males and females are equally affected and there is no significant racial preference except in the Asian population which has a lower occurrence.

Signs and Symptoms

Children with Wilms’ tumor may demonstrate some or all of the following:

  1. A mass may be felt in the child’s abdomen during bathing or dressing, or the pediatrician may detect it during a routine physical examination.
  2. Fever
  3. Blood in the urine
  4. Reduced appetite
  5. Weight loss
  6. High blood pressure
  7. Constipation
  8. Stomach pain
  9. Nausea
  10. Vomiting

Possible Causes

Wilms’ tumor appears to result from changes in one or more genes. Genes program cells to develop, grow, mature, and die. Cancer results when these changes arise in genes that control growth, allowing cells to multiply without restraint. The gene that causes Wilms’ tumor is WT-1. Certain birth defects or syndromes are associated with 10-15% of Wilms’ tumor cases, especially WAGR syndrome, Beckwith-Wiedemann syndrome, and Denys-Drash syndrome. Other associated anomalies and syndromes include aniridia (absence of the iris in the eye), genitourinary abnormalities, hemi-hypertrophy, Perlman syndrome, and Simpson-Golabi-Behmel syndrome. It has also been suggested that children with fathers employed as a welder or mechanics are at increased risk of Wilms, although evidence is not conclusive.

Diagnosis

Wilms’ tumor can be diagnosed through physical exam, blood and urine tests, as well as imaging studies. An abdominal mass is often, although not always, felt on physical examination. Laboratory evaluation includes a complete blood count (CBC) with platelets, electrolytes, liver function tests, renal function tests, serum calcium, and a urinalysis. Diagnostic imaging should include an abdominal ultrasound and CT (computed tomography) or Magnetic Resonance imaging (MRI) to identify the origin of the mass and to evaluate the status of the other kidney, as well as to evaluate for spread to the liver and near-by blood vessels. A chest X-ray, along with a chest CT should be obtained to check for lung involvement. An echocardiogram is also useful for detecting the presence of tumor in the heart.

Treatment

Standard treatment for Wilms’ Tumor includes the combination of surgery and chemotherapy. The treatment your child undergoes largely depends on the stage of the cancer and the appearance of the tumor under the microscope. The first step in the treatment is surgical removal of the primary tumor at diagnosis. Samples from the kidney, lymph nodes and any tissues that appear abnormal are taken and examined by microscope to identify cancer cells. Chemotherapy typically follows removal of the affected kidney and involves the combination of several agents to kill any residual cancer cells. The duration and timing of chemotherapy treatments may vary. Finally, radiation therapy is often given to patients with aggressive tumors.

Prognosis

The overall cure rate for patients with Wilms’ tumor approaches 80-90%. Once cured, patients can live healthy, productive lives with normal life spans. It is critical that patients be monitored for long-term effects of chemotherapy and radiation which may include: growth delay, cognitive deficits, impaired fertility, and secondary cancers.

Connect with other parents

In the spirit of community and support, Madisons Foundation offers the unique service of connecting parents of children with rare diseases. If you would like to be connected to other parents of children with this disease, please fill out this brief form.

Weblinks

MedlinePlus
Great central website with lots of useful links.

American Cancer Society:
Informative website from a reputable national organization.

MayoClinic.com:
Good clinical resource.

National Cancer Institute:
http://www.meb.uni-bonn.de/cancer.gov/CDR0000062789.html
Both of these sites are excellent with lots of extensive information.

Pediatric Oncology Resource Center
Specific to the needs of kids with cancer.

Curesurch.org
Information on chemotherapy for parents.

Google Search for Wilms' Tumor (WT)

References and Sources

Emedicine: http://www.emedicine.com/med/topic3093.htm National Wilms Tumor Society: http://www.nwtsg.org/