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Most patients with Wilms tumor have an asymptomatic abdominal mass,
but potentially life-threatening conditions include hypertension, gross
hematuria, and an acute abdomen.

Current Evidence
There are several different causes of renal tumors in children, some malignant and
some benign. Wilms tumor, the most common pediatric renal tumor, arises from
embryonic renal blastemal cells (see Fig. 98.2 ). Overgrowth syndromes such as
Beckwith–Wiedemann, Soto syndrome, hemihypertrophy, and Denys–Drash
syndrome predispose to embryonal tumors such as Wilms’. There are 500 cases of
Wilms’ diagnosed annually in United States with peak incidence between the ages
of 2 and 3. Eighty percent of cases are diagnosed by age 5.



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