RENAL ANGIOMYOLIPOMA IN ASSOCIATION WITH PULMONARY LYMPHANGIOLEIOMYOMATOSIS - FORME-FRUSTE OF TUBEROUS SCLEROSIS

From 1976 to 1989, 27 patients with pulmonary lymphangioleiomyomatosis were seen at our institution. Twenty-five had no signs or symptoms of tuberous sclerosis, 21 of these had abdominal imaging or surgical exp loration to make the diagnosis of renal angiomyolipoma in seven patien ts, all female. Three presented with symptoms secondary to renal disea se, three with pulmonary manifestations, and one with both. Renal dise ase was bilateral in four patients; four patients underwent nephrectom y and two tumors were embolized. All patients experienced spontaneous pneumothoraces during the course of their disease. There was one respi ratory death. It is our belief that any patient presenting with angiom yolipoma and a history of pulmonary problems should be evaluated for p ulmonary lymphangioleiomyomatosis with a limited computed tomography s can of the chest at the time of abdominal imaging. The surgical treatm ent of angiomyolipoma when associated with lymphangioleiomyomatosis is predicated on the ability to adequately manage the complicating lung disease perioperatively.