HOW COMMON ARE RENAL ANGIOMYOLIPOMAS IN PATIENTS WITH PULMONARY LYMPHANGIOMYOMATOSIS

Pulmonary lymphangiomyomatosis has been associated with renal angiomyo lipoma in case reports, but the prevalence of this association has not been well documented. The objective of this study was to determine th e frequency of renal angiomyolipoma in a series of subjects with pulmo nary lymphangiomyomatosis. Eighteen consecutive patients with pulmonar y lymphangiomyomatosis were seen at a single institution between 1989 and 1994. Of these, one patient was excluded because she did not have an abdominal computed tomographic (CT) scan. We found eight out of 17 (47%) patients with pulmonary lymphangiomyomatosis to have renal angio myolipomas. These were found either at surgery or on abdominal CT scan ning, Thus, renal angiomyolipomas occur commonly in association with p ulmonary lymphangiomyomatosis. Consequently, the early detection of re nal angiomyolipoma by abdominal CT may be important, because lesions w ith dimensions larger than 4 cm may present an increased risk for comp lications related to tumor growth or hemorrhage. Serial follow-up by u ltrasonography or CT scanning is important in identifying and monitori ng high-risk patients. Prophylactic treatment (partial or total nephre ctomy) may be considered for patients with tumors that show significan t growth or other complications, such as hemorrhage.