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PredicateObject
rdf:type
lifeskim:mentions
pubmed:dateCreated
2011-9-9
pubmed:abstractText
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 ?-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.
pubmed:language
eng
pubmed:journal
pubmed:status
PubMed-not-MEDLINE
pubmed:issn
1757-790X
pubmed:author
pubmed:issnType
Electronic
pubmed:volume
2009
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:year
2009
pubmed:articleTitle
Pulmonary lymphangioleiomyomatosis: unusual manifestations of multiple large pulmonary nodules with retroperitoneal lymph node involvement mimicking metastatic malignancy.
pubmed:affiliation
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.
pubmed:publicationType
Journal Article