Bilateral renal cell carcinoma in a patient with tuberous sclerosis

A case of pure bilateral renal cell carcinoma (RCC) in a 21-year-old female diagnosed as having tuberous sclerosis is reported. She underwent a left nephrectomy because of her loss of appetite, possibly caused by the tumor compressing her intestines. The preoperative CT scan showed the presence of adipose tissue in bilateral renal tumors, which is highly suggestive of angiomyolipoma (AML). Histological examination, however, revealed no area or component of the tumor with features characteristic of AML.