Clear cell sarcoma of the kidney


  • Highly malignant mesenchymal tumor of pediatric kidney, also termed bone-metastasizing renal tumor of childhood for its striking predilection to metastasize to bone

Clinical features


  • 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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