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The characteristics of RCC\u2010affected children and their tumors, the influence of treatment, and outcome have so far not been studied in a nonselected, population\u2010based cohort.<\/jats:p><\/jats:sec><jats:sec><jats:title>METHODS.<\/jats:title><jats:p>A retrospective analysis was undertaken of RCC patients less than 16 years old reported to the German Childhood Cancer Registry and to the Kiel Paediatric Tumor Registry from 1980 to 2005.<\/jats:p><\/jats:sec><jats:sec><jats:title>RESULTS.<\/jats:title><jats:p>Forty\u2010nine RCC in children (24 boys, 25 girls) with a median age of 10.6 years were identified. In about every third child possibly RCC\u2010related underlying disorders (tuberous sclerosis, neuroblastoma, teratoma with chemotherapy, Saethre\u2010Chotzen syndrome, chronic renal failure) or related diseases in their family were found. The pathologic subtypes were papillary in 16 (33%), translocation type in 11 (22%), unclassified in 8 (16%), and rarely clear\u2010cell (n = 3) or others. Thirty\u2010four (69%) patients had localized RCC, 8 (16%) patients regional lymph node metastases, and 4 (8%) patients distant metastases. Event\u2010free survival and overall survival rates at 5 years were 96% for localized RCC, 69% and 75% for regional lymph node\u2010positive, 25% and 33% for distant metastatic RCC, respectively. Two of 4 patients with distant metastases received immunotherapy combined with chemotherapy and surgery, both are alive, 1 of them disease\u2010free for 6.9 years.<\/jats:p><\/jats:sec><jats:sec><jats:title>CONCLUSIONS.<\/jats:title><jats:p>Pediatric RCCs are predominantly localized diseases. Children with RCC frequently suffer underlying disorders. Survival rates in localized and regional lymph node\u2010positive cases are favorable. Because of the rarity of RCC in childhood, an international study is necessary. Cancer 2006. \u00a9 2006 American Cancer Society.<\/jats:p><\/jats:sec>","DOI":"10.1002\/cncr.22346","type":"journal-article","created":{"date-parts":[[2006,11,17]],"date-time":"2006-11-17T15:57:56Z","timestamp":1163779076000},"page":"2906-2914","source":"Crossref","is-referenced-by-count":112,"title":["Population\u2010based study of renal cell carcinoma in children in Germany, 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