Renal involvement in tuberous sclerosis

Three patients are described with renal angiomyolipoma without accompaning central nervous system involvement. Each patient presented in a different manner; the first with signs and symptoms of renal insufficiency, the second with gross hematuria, and the third with flank pain. All patients had replacement of renal parenchyma by hamartomatous tumors composed of blood vessels, adipose tissue and smooth muscle cells. The first case is the fifth report of the development of renal failure in tuberous sclerosis. In the latter two cases, intravenous pyelograms suggested polycystic kidney disease but renal arteriography was diagnostic of diffuse angiomyolipomas. Malignant transformation of these tumors is rare, but hemorrhagic complications following renal biopsy is a major problem in these patients.