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Highly malignant mesenchymal tumor of pediatric kidney, also termed bone-metastasizing renal tumor of childhood for its striking predilection to metastasize to bone
Accounts for 3% of pediatric renal tumors, with approximately 20 new cases diagnosed annually in the United States
Peak incidence between 12 and 36 months of age, with a sharp decline after that period
Male-to-female ratio, 2:1 to 3:1
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