Abstract

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• A 42-year-old woman with tuberous sclerosis (TS) presented with acute right-sided flank pain. Imaging studies demonstrated numerous bilateral renal angiomyolipomas (AML), the largest located within the right upper pole, extending into the venous system to the level of the infra-hepatic inferior vena cava (IVC). Intravascular extension of AML is quite rare, however, may potentially result in fatal complications if not appropriately treated. We present a case report and a description of the surgical management, and provide a review of the literature concerning this rare finding.