A 49-year-old woman with tuberous sclerosis visited our hospital complaining of left abdominal pain. She had been diagnosed with bilateral renal angiomyolipoma (AML) when she was 32 years old and had been on maintenance hemodialysis since the age of 44. Magnetic resonance imaging showed bilateral giant renal AML and hemorrhage in the AML of the left kidney. Transthoracolumbar left nephrectomy was performed in March 2008. The resected specimen weighed 3.2 kg. The histological diagnosis was consistent with AML. In addition, the Japanese literature on giant renal AML was reviewed and discussed.
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