Open AccessPulmonary lymphangioleiomyomatosis: unusual manifestations of multiple large pulmonary nodules with retroperitoneal lymph node involvement mimicking metastatic malignancy
Author(s) -
Hye-Jung Kim,
ChangHoon Lee,
Yong-A Kim,
Daehee Han,
Hanlim Moon,
Hey Won Cheon,
Hee Soon Chung,
Deog Kyeom Kim
Publication year - 2009
Publication title -
bmj case reports
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.231
H-Index - 26
ISSN - 1757-790X
DOI - 10.1136/bcr.02.2009.1620
Subject(s) - medicine , malignancy , lymphangioleiomyomatosis , lung , pathology , wedge resection , radiology , solitary pulmonary nodule , tuberous sclerosis , surgery , resection
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.