Two different types of infantile renal cell carcinomas associated with tuberous sclerosis

Renal cell carcinoma (RCC) associated with tuberous sclerosis (TSC) has been reported in adult patients; however, this association is rare in children. We have encountered 2 different types of RCCs in infants who developed TSC later in childhood. The pathologic diagnoses were papillary RCC in one infant and a clear cell–type RCC in the other. Gene analysis showed that both the patients had a mutation in TSC2 gene on chromosome 16.