Source:http://linkedlifedata.com/resource/pubmed/id/21904573
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rdf:type | |
lifeskim:mentions | |
pubmed:dateCreated |
2011-9-9
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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.
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pubmed:language |
eng
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pubmed:journal | |
pubmed:status |
PubMed-not-MEDLINE
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pubmed:issn |
1757-790X
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pubmed:author | |
pubmed:issnType |
Electronic
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pubmed:volume |
2009
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:year |
2009
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pubmed:articleTitle |
Pulmonary lymphangioleiomyomatosis: unusual manifestations of multiple large pulmonary nodules with retroperitoneal lymph node involvement mimicking metastatic malignancy.
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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.
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pubmed:publicationType |
Journal Article
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