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Renal angiomyolipomas in patients with tuberous sclerosis can be associated with significant morbidity, mostly related to complications from bleeding. We describe a patient with tuberous sclerosis and massive bilateral renal angiomyolipomas (total tumor burden 5500 g) who presented with acute right renal hemorrhage. She was treated with right renal artery embolization followed immediately by right nephrectomy and left partial nephrectomy. The patient had a creatinine nadir of 1.3 mg/dL postoperatively. We demonstrate that nephron-sparing surgery is feasible, even in the setting of very large angiomyolipomas, such as the one presented here, currently the largest such tumor by weight reported.
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