Bilateral angiomyolipomas and renal cell carcinoma in polycystic kidney

A case is presented of a twenty-eight-year-old man in whom renal failure developed at age twenty-four from polycystic kidney disease known to be present since childhood. He also had cutaneous manifestations of the tuberous sclerosis complex. Intrarenal hemorrhage led to bilateral nephrectomy. Microscopic examination disclosed typical polycystic disease and multiple angiomyolipomas in each kidney. In addition several renal cell carcinomas of oncocytic, papillary, and clear cell type were found. Review of the literature disclosed the uncommon coexistence of any two of these lesions and did not uncover any reported case of the simultaneous existence of all three.