Abstract
Renal involvement is common in tuberous sclerosis. Angiomyolipomas and cysts are found in ∼50 and 30% of patients respectively, and often occur together. Tuberous sclerosis also appears to be associated with a small but increased risk of renal cell carcinoma. Recent studies have begun to elucidate the molecular genetic mechanisms underlying the renal manifestations of this systemic autosomal dominant disorder.

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