Four cases of hemangioleiomyolipoma of the kidney are presented, 3 of which were diagnosed in non tuberous sclerosis patients. In 1 case diagnosis was made preoperatively and in another case it was made intraoperatively, allowing for preservation of functioning renal parenchyma. The second successful kidney transplant in a patient with tuberous sclerosis and renal failure is reported. One cannot always differentiate a hemangiomyolipoma from an adenocarcinoma. However, if the classical angiographic findings of sacculated pseudo aneurysms supplied by the interlobular and interlobar arteries are present non operative observation or limited surgery with preservation of renal tissue is possible. Also, knowledge of the gross pathologic appearance and the syndrome of tuberous sclerosis will allow one to make a preoperative or intraoperative diagnosis with confidence.
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