Wilms Tumor & Other Childhood Kidney Tumors

At a Glance

  • Wilms tumor usually occurs in children younger than five years old.
  • Wilms tumor is a curable disease in the majority of affected children.
  • Since the 1980s, the 5-year survival rate for Wilms tumor has been consistently above 90 percent.
  • Approximately 500 cases are diagnosed in the United States each year.

About Wilms Tumor

Wilms tumor and other childhood kidney tumors are diseases in which malignant (cancer) cells form in the tissues of the kidney. Wilms tumor usually occurs in children younger than 5 years old. Wilms tumor is a curable disease in the majority of affected children. Approximately 500 cases are diagnosed in the United States each year. Since the 1980s, the 5-year survival rate for Wilms tumor has been consistently above 90 percent with various treatment options. In Wilms tumor, one or more tumors may be found in one or both kidneys. There are two kidneys, one on each side of the backbone, above the waist. Tiny tubules in the kidneys filter and clean the blood, taking out waste products and making urine. The urine passes from each kidney through a long tube called a ureter into the bladder. The bladder holds the urine until it is passed from the body. Wilms tumor may spread to the lungs, liver, or nearby lymph nodes. Nephroblastomatosis is a condition in which abnormal tissue grows on the outer part of one or both kidneys. Children with this condition are at risk for developing a type of Wilms tumor that grows quickly.

Other Kidney Tumors

Other childhood kidney tumors, which are diagnosed and treated in different ways, include:
  • Clear cell sarcoma of the kidney is a type of kidney tumor that may spread to the lung, bone, brain, and soft tissue.
  • Rhabdoid tumor of the kidney is a type of cancer that occurs mostly in infants and young children. It grows and spreads quickly, often to the lungs and brain.
  • Neuroepithelial tumors of the kidney are rare and usually occur in young adults. They grow and spread quickly.
  • Desmoplastic small round cell tumor of the kidney is a rare soft tissue sarcoma.
  • Cystic partially differentiated nephroblastoma is a very rare type of Wilms tumor made up of cysts.
  • Renal cell carcinoma is rare in children or in adolescents younger than 15 years of age. However, it is much more common in adolescents between 15 and 19 years of age. Renal cell carcinomas can spread to the lungs, bones, liver, and lymph nodes.
  • Mesoblastic nephroma is a tumor of the kidney that is usually diagnosed within the first year of life and can usually be cured. One type of mesoblastic nephroma may appear on an ultrasound exam before birth or may occur within the first 3 months after the child is born. Mesoblastic nephroma occurs more often in males than females.
  • Primary renal synovial sarcoma is a rare tumor of the kidney and is most common in young adults.
  • Anaplastic sarcoma of the kidney is a rare tumor that is most commonly found in children or adolescents younger than 15 years of age. Anaplastic sarcoma of the kidney often spreads to the lungs, liver, or bones. There is no standard treatment for anaplastic sarcoma.

Risk Factors

Anything that increases the risk of getting a disease is called a risk factor. Wilms tumor may be part of a genetic syndrome that affects growth or development. A genetic syndrome is a set of symptoms or conditions that occur together and is usually caused by abnormal genes. Certain birth defects can also increase a child’s risk for developing Wilms tumor. The following genetic syndromes and birth defects have been linked to Wilms tumor:
  • WAGR syndrome (Wilms tumor, aniridia, abnormal genitourinary system, and mental retardation).
  • Beckwith-Wiedemann syndrome.
  • Hemihypertrophy (abnormally large growth of one side of the body or a body part).
  • Denys-Drash syndrome.
  • Cryptorchidism.
  • Hypospadias.
Children with these genetic syndromes and birth defects should be screened for Wilms tumor every three months until age 8. An ultrasound test may be used for screening. Having certain conditions may be associated with renal cell carcinoma. Renal cell carcinoma may be related to the following conditions:
  • Von Hippel-Lindau disease (an inherited condition that causes abnormal growth of blood vessels).
  • Tuberous sclerosis (an inherited disease marked by noncancerous fatty cysts in the kidney).
  • Neuroblastoma and/ or sickle cell disease.

Symptoms

Possible signs of Wilms tumor and other childhood kidney tumors include:
  • A lump, swelling, or pain in the abdomen.
  • Blood in the urine.
  • Fever for no known reason.
If you have symptoms, increased risk factors or have recently been diagnosed with Wilms Kidney Tumors, contact us to request an appointment with Diablo Valley Oncology’s physicians.