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Wilms tumor is the most common malignant tumor of the kidney in childhood, affecting about 1 in 10,000 children; about 1 in 20 childhood tumors in the United States is a Wilms tumor. It affects boys and girls equally; the peak age of diagnosis is three to four years. It is more common in African-American children than whites or Asians.
Wilms tumors are usually discovered as an abdominal mass detected on a routine physical or illness exam, but may present with abdominal symptoms such as chronic abdominal pain, weight loss, vomiting, bloody urine (hematuria) or hypertension. Children with aniridia (a rare congenital absence of the iris), hemihypertrophy, or Beckwith-Weidemann syndrome (large birthweight, hemihypertrophy, large tongue and liver, and predisposition to Wilms tumor) are at increased risk for the tumor and are screened with ultrasound examination of the kidneys every 3 months until about age 7, followed by semi-annual abdominal physical exam.
Severity of Wilms tumor is graded in 5 stages. Treatment involves removal, chemotherapy, and possible radiation. Cure rates are generally very good, nearly 90% for disease localized to the kidney and 80% for metastatic disease.