A Case of Pulmonary Lymphangioleiomyomatosis Developing 3 Years After Complete Surgical Removal of Abdominal Lymphangioleiomyoma: Case Report and Review Article

Abstract: Pulmonary lymphangioleiomyomatosis (LAM) is a rare disease that affects almost exclusively women, usually in childbearing age. LAM is usually mistaken for other common pulmonary diseases like asthma, bronchitis, or chronic interstitial lung diseases. We present a woman who developed pulmonary LAM after complete surgical removal of abdominal lymphangioleiomyoma 3 years earlier. Chest computed tomography at time of surgery did not show evidence of pulmonary involvement. LAM cells have the ability to proliferate and migrate to distant sites. Thus, pulmonary LAM is thought by many authors to originate from remote sites such as abdominal lymphangioleiomyomas and angiomyolipomas. Few reports in the past have documented the occurrence of pulmonary LAM 2 to 3 years after the excision of abdominal LAM lesions; however, the excision was not complete in these cases. Our case report may suggest that complete surgical removal of abdominal lymphangioleiomyoma may not prevent the development of pulmonary LAM or halt its progression. The issue of occurrence of pulmonary LAM after complete excision of extrapulmonary LAM lesions needs to be further elucidated to better define the clinical behavior of this disease.