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Abstract:The present study aimed to assess the clinicopathological characteristics and the prognosis of renal cell carcinoma (RCC) in children. We retrospectively reviewed the medical records of 11 Korean children (7 boys and 4 girls) undergoing radical nephrectomy for RCC between September 1997 and January 2008 at our institution. Mean age at diagnosis was 12.7 years. The presenting clinical signs and symptoms included gross hematuria (55%), abdominal mass (45%) and abdominal pain (45%). Two (18%) of the cases were discovered incidentally. Five (45%) were conventional clear cell, four (36%) were papillary, one was a chromophobe and one was a translocation carcinoma. Seven children were T1N0M0, three were T2N0M0 and one child was T1N1M0. While one patient with T2 disease died of disease 89 months postoperatively, the remaining 10 patients, including a patient with regional nodal disease who received adjuvant immunochemotherapy, survived without evidence of recurrence at a mean follow-up of 6.8 years. Our data suggest that the clinical presentation, pathological characteristics and clinical behavior of pediatric RCC are different from those of adult RCC.