Bilateral massive renal angiomyolipoma concurrent with oncocytoma in tuberous sclerosis complex associated with pulmonary lymphangioleiomyomatosis.

We describe a patient with tuberous sclerosis complex and massive bilateral renal angiomyolipomas (AMLs) in association with pulmonary lymphangioleiomatosis, who experienced hemorrhagic shock because of rupture of the left renal AML. She was treated with transarterial embolization, followed by bilateral nephrectomy. The total tumor burden weighed 5800 g, making it the largest AML by weight in published reports. The histopathologic examination revealed concurrent oncocytomas with AMLs in the right kidney, indicating a possible shared mechanism in the pathogenesis of these two disorders. This case also represents a rare, but significant, overlap between renal AML, pulmonary lymphangioleiomatosis, and tuberous sclerosis complex.