Tuberous sclerosis, a genetically transmitted multisystem tem neurocut
aneous disorder, is associated with renal lesions in 50% of cases. The
expected lesions are angiomyolipomas or renal cysts; renal cell carci
noma has been encountered in adults who have tuberous sclerosis. but i
s very rare in children. The authors report the case of a 5 year-old g
irl with tuberous sclerosis for whom atypical computed tomography find
ings led to the diagnosis of renal carcinoma at an early age. This exp
erience suggests that children with tuberous sclerosis may need earlie
r screening. (C) 1996 by W.B. Saunders Company