A Clinicopathological Analysis of Pulmonary Lymphangioleiomyomatosis
Background and objective Pulmonary lymphangioleiomyomatosis (PLAM) is a rare tumor with unique clinicopathological features. The aim of this study is to investigate the clinicopathological features, the diagnosis and differential diagnosis of pulmonary lymphangioleiomyomatosis. Methods Three cases o...
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Chinese Anti-Cancer Association; Chinese Antituberculosis Association
2011-04-01
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Online Access: | http://www.lungca.org/index.php?journal=01&page=article&op=viewFile&path[]=10.3779%2Fj.issn.1009-3419.2011.04.14&path[]=3740 |
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doaj-8de747527c7248188a04f72a4c164c702020-11-25T01:22:17ZzhoChinese Anti-Cancer Association; Chinese Antituberculosis AssociationChinese Journal of Lung Cancer1009-34191999-61872011-04-01144378382A Clinicopathological Analysis of Pulmonary LymphangioleiomyomatosisJun GAOPeiju ZHUShangfu ZHANGSha ZHAOChangli LUHuijiao CHENBackground and objective Pulmonary lymphangioleiomyomatosis (PLAM) is a rare tumor with unique clinicopathological features. The aim of this study is to investigate the clinicopathological features, the diagnosis and differential diagnosis of pulmonary lymphangioleiomyomatosis. Methods Three cases of PLAM were analyzed by light microscopy, immunohistochemistry and their clinical data, and the relative literatures were reviewed. Results Three cases of patients suffered from PLAM were the women in their reproductive aged, from 27 years to 45 years (mean 37.7 years), two cases of the HRCT showed bilateral diffuse cystic airspaces changed, and one case was the pneumothorax. The histopathological examination revealed the tumor was composed of the variably sized cystic spaces are lined by plaque-like or nodular aggregates of endothelial cells and the hyperplasia, smooth-muscle-like spindle cells which was along with the bronchi and the vessels. The immunohistochemistry showed that Des, Caldes, SMA, MSA, HMB-45, CD63, Vim, ER and PR were positive in the hyperplasia spindle cells, and there was no expression of MRAT-1. The FVIII, CD34 were positive in the capillary endothelial cells, and the D2-40 was positive in the lymphatic vessels. All the patients were alive without the recrudescence of the PLAM since the diagnosis, about 3 months to 25 months, and there was no LAM in their other systems. Conclusion The most significant histopathological feature of pulmonary lymphangioleiomyomatosis was the progressive invasion of smooth muscle cells into the lymphatic vessels, and the blood vessels. The majority of the cases occur in the lungs of the women in the predominantly premenopausal and middle-age. It is a poor prognosis due to the progressive respiratory failure.http://www.lungca.org/index.php?journal=01&page=article&op=viewFile&path[]=10.3779%2Fj.issn.1009-3419.2011.04.14&path[]=3740Lung neoplasmsLymphangioleiomyomatosisImmunohistochemistryDiagnosis |
collection |
DOAJ |
language |
zho |
format |
Article |
sources |
DOAJ |
author |
Jun GAO Peiju ZHU Shangfu ZHANG Sha ZHAO Changli LU Huijiao CHEN |
spellingShingle |
Jun GAO Peiju ZHU Shangfu ZHANG Sha ZHAO Changli LU Huijiao CHEN A Clinicopathological Analysis of Pulmonary Lymphangioleiomyomatosis Chinese Journal of Lung Cancer Lung neoplasms Lymphangioleiomyomatosis Immunohistochemistry Diagnosis |
author_facet |
Jun GAO Peiju ZHU Shangfu ZHANG Sha ZHAO Changli LU Huijiao CHEN |
author_sort |
Jun GAO |
title |
A Clinicopathological Analysis of Pulmonary Lymphangioleiomyomatosis |
title_short |
A Clinicopathological Analysis of Pulmonary Lymphangioleiomyomatosis |
title_full |
A Clinicopathological Analysis of Pulmonary Lymphangioleiomyomatosis |
title_fullStr |
A Clinicopathological Analysis of Pulmonary Lymphangioleiomyomatosis |
title_full_unstemmed |
A Clinicopathological Analysis of Pulmonary Lymphangioleiomyomatosis |
title_sort |
clinicopathological analysis of pulmonary lymphangioleiomyomatosis |
publisher |
Chinese Anti-Cancer Association; Chinese Antituberculosis Association |
series |
Chinese Journal of Lung Cancer |
issn |
1009-3419 1999-6187 |
publishDate |
2011-04-01 |
description |
Background and objective Pulmonary lymphangioleiomyomatosis (PLAM) is a rare tumor with unique clinicopathological features. The aim of this study is to investigate the clinicopathological features, the diagnosis and differential diagnosis of pulmonary lymphangioleiomyomatosis. Methods Three cases of PLAM were analyzed by light microscopy, immunohistochemistry and their clinical data, and the relative literatures were reviewed. Results Three cases of patients suffered from PLAM were the women in their reproductive aged, from 27 years to 45 years (mean 37.7 years), two cases of the HRCT showed bilateral diffuse cystic airspaces changed, and one case was the pneumothorax. The histopathological examination revealed the tumor was composed of the variably sized cystic spaces are lined by plaque-like or nodular aggregates of endothelial cells and the hyperplasia, smooth-muscle-like spindle cells which was along with the bronchi and the vessels. The immunohistochemistry showed that Des, Caldes, SMA, MSA, HMB-45, CD63, Vim, ER and PR were positive in the hyperplasia spindle cells, and there was no expression of MRAT-1. The FVIII, CD34 were positive in the capillary endothelial cells, and the D2-40 was positive in the lymphatic vessels. All the patients were alive without the recrudescence of the PLAM since the diagnosis, about 3 months to 25 months, and there was no LAM in their other systems. Conclusion The most significant histopathological feature of pulmonary lymphangioleiomyomatosis was the progressive invasion of smooth muscle cells into the lymphatic vessels, and the blood vessels. The majority of the cases occur in the lungs of the women in the predominantly premenopausal and middle-age. It is a poor prognosis due to the progressive respiratory failure. |
topic |
Lung neoplasms Lymphangioleiomyomatosis Immunohistochemistry Diagnosis |
url |
http://www.lungca.org/index.php?journal=01&page=article&op=viewFile&path[]=10.3779%2Fj.issn.1009-3419.2011.04.14&path[]=3740 |
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