title: Pulmonary lymphangioleiomyomatosis: unusual manifestations of multiple large pulmonary nodules with retroperitoneal lymph node involvement mimicking metastatic malignancy.
abstract: Pulmonary lymphangioleiomyomatosis (LAM) is a rare, idiopathic disorder that predominantly affects the lung parenchyma of women of childbearing age. While the characteristic radiographic finding of pulmonary LAM consists of multiple well-defined thin-walled cysts, we describe a very unusual case of pulmonary LAM with multiple bilateral large pulmonary nodules and retroperitoneal involvement mimicking metastatic malignancy. A 48-year-old woman who had never smoked with a history of bilateral pneumothorax presented with progressive exertional dyspnea and abdominal discomfort. Imaging studies revealed multiple enlarged retroperitoneal lymph nodes, ascites and bilateral multiple large pulmonary nodules ranging from 3 to 18 mm in diameter. Exploratory laparoscopic surgery for intra-abdominal lesions and video-assisted thoracoscopic wedge resection of lung nodules were carried out to rule out metastatic malignancy. Pathology showed benign looking smooth muscle cell proliferation and immunoreactivity for α-smooth muscle actin and HMB-45 in both specimens. After treatment with GnRH antagonist, the patient was well over a 6-month period without evidence of disease progression.
authors: Division of Pulmonology and Critical Care Medicine, Department of Internal Medicine, Seoul National University Bundang Hospital, 166 Gumi-ro Bundang-gu, Seongnam-si Geonggi-do, 463-707, Republic of Korea.
authors: Hye-Jung
authors: HJ
authors: Kim
authors: Chang-Hoon
authors: CH
authors: Lee
authors: Yong-A
authors: YA
authors: Kim
authors: Daehee
authors: D
authors: Han
authors: Hyeon Jong
authors: HJ
authors: Moon
authors: Hey Won
authors: HW
authors: Cheon
authors: Hee Soon
authors: HS
authors: Chung
authors: Deog Kyeom
authors: DK
authors: Kim