Learn About Wilms Tumor

What is the definition of Wilms Tumor?

Wilms tumor (WT) is a type of kidney cancer that occurs in children.

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What are the alternative names for Wilms Tumor?

Nephroblastoma; Kidney tumor - Wilms

What are the causes of Wilms Tumor?

WT is the most common form of childhood kidney cancer. The exact cause of this tumor in most children is unknown.

A missing iris of the eye (aniridia) is a birth defect that is sometimes associated with WT. Other birth defects linked to this type of kidney cancer include certain urinary tract problems and swelling of one side of the body, a condition called hemihypertrophy.

It is more common among some siblings and twins, which suggests a possible genetic cause.

The disease occurs most often in children about 3 years old. More than 90% of cases are diagnosed before 10 years of age. In rare cases, it is seen in children older than 15 years of age, and in adults.

What are the symptoms of Wilms Tumor?

Symptoms may include any of the following:

  • Abdominal pain
  • Abnormal urine color
  • Constipation
  • Fever
  • General discomfort or uneasiness (malaise)
  • High blood pressure
  • Increased growth on only one side of the body
  • Loss of appetite
  • Nausea and vomiting
  • Swelling in the abdomen (abdominal hernia or mass)
  • Sweating (at night)
  • Blood in urine (hematuria)
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What are the current treatments for Wilms Tumor?

If your child is diagnosed with WT, do not prod or push on the child's belly area. Use care during bathing and handling to avoid injury to the tumor site.

The first step in treatment is to stage the tumor. Staging helps the provider determine how far the cancer has spread and to plan for the best treatment. Surgery to remove the tumor is planned as soon as possible. Surrounding tissues and organs may also need to be removed if the tumor has spread.

Radiation therapy and chemotherapy will often be started after surgery, depending on the stage of the tumor.

Chemotherapy given before the surgery is also effective in preventing complications.

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What is the outlook (prognosis) for Wilms Tumor?

Children whose tumor has not spread have a 90% cure rate with appropriate treatment. Prognosis is also better in children younger than 2 years of age.

What are the possible complications of Wilms Tumor?

The tumor may become quite large, but usually remains self-enclosed. Spread of the tumor to the lungs, lymph nodes, liver, bone, or brain is the most worrisome complication.

High blood pressure and kidney damage may occur as the result of the tumor or its treatment.

Removal of WT from both kidneys may affect kidney function.

Other possible complications of the long-term treatment of WT may include:

  • Heart failure
  • Secondary cancer elsewhere in the body that develops after the treatment of first cancer
  • Short height
When should I contact a medical professional for Wilms Tumor?

Call your child's provider if:

  • You discover a lump in your child's abdomen, blood in the urine, or other symptoms of WT.
  • Your child is being treated for this condition and symptoms get worse or new symptoms develop, mainly cough, chest pain, weight loss, or persistent fevers.
How do I prevent Wilms Tumor?

For children with a known high risk for WT, screening using ultrasound of the kidneys or prenatal genetic analysis may be suggested.

Kidney anatomy
Wilms tumor
What are the latest Wilms Tumor Clinical Trials?
Renal Tumors Classification, Biology, and Banking Study

Summary: This research trial studies kidney tumors in younger patients. Collecting and storing samples of tumor tissue, blood, and urine from patients with cancer to study in the laboratory may help doctors learn more about changes that occur in deoxyribonucleic acid (DNA) and identify biomarkers related to cancer.

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A Phase I Study of Vorinostat in Combination With Vincristine, Irinotecan, and Temozolomide in Children, Adolescents, and Young Adults With Relapsed or Refractory Solid Tumors and CNS Malignancies

Summary: Investigators are testing new experimental drug combinations such as the combination of vorinostat, vincristine, irinotecan, and temozolomide in the hopes of finding a drug that may be effective against tumors that have come back or that have not responded to standard therapy. The goals of this study are: To find the highest safe dose of vorinostat that can be given together with vincristine, irin...

What are the Latest Advances for Wilms Tumor?
Congenital Teratocarcinosarcoma With CTNNB1 Gene Mutation Presenting as an Ocular Mass.
Wilms' tumor 1 peptide-loaded dendritic cell vaccination in patients with relapsed or refractory acute leukemia.
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Dimethylarginines in Children after Anti-Neoplastic Treatment.
Who are the sources who wrote this article ?

Published Date: April 29, 2022
Published By: Todd Gersten, MD, Hematology/Oncology, Florida Cancer Specialists & Research Institute, Wellington, FL. Review provided by VeriMed Healthcare Network. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.

What are the references for this article ?

National Cancer Institute website. Wilms tumor and other childhood kidney tumors treatment (PDQ) - health professional version. www.cancer.gov/types/kidney/hp/wilms-treatment-pdq. Updated April 5, 2022. Accessed August June 6, 2022.

Ritchey ML, Cost NG, Shamberger RC. Pediatric urologic oncology: renal and adrenal. In: Partin AW, Dmochowski RR, Kavoussi LR, Peters CA, eds. Campbell-Walsh-Wein Urology. 12th ed. Philadelphia, PA: Elsevier; 2021:chap 53.

Weiss RH, Jaimes EA, Hu SL. Kidney cancer. In: Yu ASL, Chertow GM, Luyckx VA, Marsden PA, Skorecki K, Taal MW, eds. Brenner and Rector's The Kidney. 11th ed. Philadelphia, PA: Elsevier; 2020:chap 41.