Background: Renal cell carcinoma (RCC) is a rare disease in children and adolescents. This study aimed to review epidemiologic characteristics and survival for pediatric RCC patients using a large, population-based database.
Procedure: The California Cancer Registry (CCR) was reviewed from 1988 to 2004. All cases of RCC in patients younger than 21 years were identified and annual age-adjusted incidence rates were determined for the overall population and subdivided by ethnicity. Tumors were classified by stage and grade, and actuarial mortality rates were calculated.
Results: From 1988 to 2004, 43 cases of RCC were identified in patients younger than 21 years, accounting for 4.3% of all renal tumors in this age group. The overall annual age-adjusted incidence was 0.01/100,000 with the tumor more common in non-Hispanic blacks (0.03/100,000) compared to non-Hispanic whites (0.01/100,000), Hispanics (<0.01/100,000), and non-Hispanic Asians/Pacific Islanders (<0.01/100,000). The mean age at presentation was 15.4 years (SD 4.03, SE 0.615). RCC was identified more frequently in females (58.14%). At the time of presentation, 53.49% of tumors were localized, 20.93% were regionally advanced, and 25.58% were metastatic. The observed actuarial survival at 5 and 10 years was 61% (+/-15.7%).
Conclusion: Pediatric RCC is an uncommon and aggressive tumor that occurs most frequently in children in the second decade of life, more often in females and blacks. The epidemiological characteristics of this tumor differ from adult RCC and Wilms tumor, suggesting its distinctive biology and potential need for alternative treatment strategies.
(c) 2008 Wiley-Liss, Inc.