Wilms Tumour

Statistics

  • In the US, an estimate of 500 to 600 children below the age of 15 are diagnosed with Wilms tumour (also known as nephroblastoma) each year.
  • The disease accounts for 5% of all childhood cancers and is the most common type of kidney cancer among children.
  • A Wilms tumour occurs most often in young children, often between the ages of 3 and 4.
  • 90% of Wilms tumours are diagnosed before age 10. 
  • As children get older, these tumours become less common but can still occur in people of any age.
  • A Wilms tumour is slightly more common in girls than boys.
  • The risk is slightly higher in black children than white children, while Asian-American children are least at risk.
  • The 5-year survival rate indicates what percentage of children live at least 5 years after being diagnosed.
    • Percent means how many out of 100.
    • The 5-year survival rate for children with a Wilms tumour is 93%.
    • However, the rate varies according to the stage of the disease.

(Cancer.Net, 2020)

Signs & Symptoms

Signs and symptoms of Wilms tumour vary widely, with some children not showing any obvious signs. But most children with Wilms tumour experience one or more of the following:

  • An abdominal mass one 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

Diagnosis

  • Children who have any syndromes or birth defects linked to Wilms tumour should be screened for the disease.
  • These children should have a physical exam and an ultrasound regularly. The ultrasound will help identify any kidney tumours before the tumour spreads to other organs.
  • According to the American Cancer Society, doctors recommend that children with a higher risk of Wilms tumour get an exam every three or four months up to the age of 8 years old.
  • Mention to your doctor if you have any family members who have had Wilms tumour. If you do, your doctor may recommend that all children in your family have regular ultrasound tests.
  • The condition is extremely rare in children who don’t have any risk factors. Therefore, tests for Wilms tumour aren’t typically done unless a child has symptoms, such as swelling in the abdomen.
  • To confirm a diagnosis of Wilms tumour, your doctor may order diagnostic tests, including:
    • blood or urine tests
    • complete blood count (CBC)
    • abdominal X-ray or ultrasound
    • CT scan or MRI

These tests will help your doctor conduct an accurate diagnosis. Once diagnosed, more tests will be done to determine how advanced the disease is. Treatment and outlook will depend on the stage of your child’s cancer.

(healthline, 2018)

Risk Factors

Factors that may increase the risk of Wilms tumour include:

  • African-American race. In the US, African-American children have a slightly higher risk of developing Wilms tumour than children of other races. Asian-American children appear to have a lower risk for developing Wilms tumour.
  • Family history of Wilms tumour. If someone in your child’s family has or has had Wilms tumour, then your child has a higher risk of developing the disease.

Wilms tumour occurs more often in children with certain abnormalities or syndromes present at birth, including:

  • Aniridia. In aniridia (an-ih-RID-e-uh), the iris — the coloured portion of the eye — develops only partially or not at all.
  • Hemihypertrophy. Hemihypertrophy (hem-e-hi-PUR-truh-fee) refers to one side of the body or a part of the body being noticeably larger than the other side.

Wilms tumour can occur as part of rare syndromes, including:

  • WAGR syndrome. This syndrome includes Wilms tumour, aniridia, genital and urinary system abnormalities, and intellectual disabilities.
  • Denys-Drash syndrome. This syndrome includes Wilms tumour, kidney disease and male pseudohermaphroditism (soo-do-her-MAF-roe-dit-iz-um), where a boy is born with testicles but may exhibit female characteristics.
  • Beckwith-Wiedemann syndrome. Children with this syndrome tend to be significantly larger than average (macrosomia). Other signs may include abdominal organs that jut out into the base of the umbilical cord, a large tongue (macroglossia), enlarged internal organs and ear abnormalities. There is also an increased risk of tumours, including a type of liver cancer called hepatoblastoma.

(Mayo Clinic, 2020)

Treatment

A doctor will come up with a treatment plan for your child. Be sure to discuss all options available for your child as well as their possible side effects.

The main types of treatment are:

  • surgery
  • chemotherapy
  • radiation therapy

Most children undergo a combination of treatments.

  • Surgery is usually the first treatment used for people in the US.
    • The goal of surgery is to remove the tumour.
  • Chemotherapy or radiation therapy. In some cases, the tumour can’t be removed because it’s too big, or the cancer may have spread to both kidneys or into the blood vessels.
    • In which case, your doctors might first use chemotherapy or radiation therapy (or both) to try to shrink the tumour before surgery.

Your child might have to undergo more chemotherapy, radiation therapy, or surgery if the tumour isn’t completely removed the first time. The specific drugs and the extent of the treatment prescribed will vary depending on your child’s condition.

(healthline, 2018)

References

  1. Cancer.Net. (2020, January). Wilms Tumor – Childhood: Statistics.
    Retrieved from Cancer.Net: https://www.cancer.net/cancer-types/wilms-tumor-childhood/statistics
  2. healthline. (2018, March 23). Wilms’ tumor.
    Retrieved from healthline: https://www.healthline.com/health/wilms-tumor#prevention
  3. Mayo Clinic. (2020, April 24). Wilms’ tumor.
    Retrieved from Mayo Clinic: https://www.mayoclinic.org/diseases-conditions/wilms-tumor/symptoms-causes/syc-20352655