Nephron-sparing nephrectomy for giant renal angiomyolipoma associated with lymphangioleiomyomatosis

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.