Renal angiomyolipomas are benign tumors known to occur sporadically and in association with genetic syndromes, including tuberous sclerosis and lymphangioleiomyomatosis. Surgical removal or radiographic embolization of angiomyolipomas larger than 4 cm is usually indicated because of an increased risk of spontaneous hemorrhage. We describe successful nephron-sparing surgery for a giant angiomyolipoma and discuss relevant management issues in a patient with lymphangioleiomyomatosis who had previously undergone bilateral pulmonary transplantation.
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