中国肺癌杂志2011，Vol.14Issue(4)：378-382,5.DOI:10.3779/j.issn.1009-3419.2011.04.14
肺淋巴管平滑肌瘤病的临床病理分析
A Clinicopathological Analysis of Pulmonary Lymphangioleiomyomatosis
高俊 1朱培菊 1张尚福 1赵莎 1鲁昌立 1陈卉娇1
作者信息
- 1. 610041,成都,四川大学华西医院病理科
- 折叠
摘要
Abstract
Background and objective Pulmonary lymphangioleiomyomatosis (PLAM) is a rare tumor with unique clinicopathological features.-lhe 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 Iined 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 maj ority 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.关键词
肺肿瘤/淋巴管平滑肌瘤病/免疫组化/诊断Key words
Lung neoplasms/ Lymphangioleiomyomatosis/ Immunohistochemistry/ Diagnosis分类
医药卫生引用本文复制引用
高俊,朱培菊,张尚福,赵莎,鲁昌立,陈卉娇..肺淋巴管平滑肌瘤病的临床病理分析[J].中国肺癌杂志,2011,14(4):378-382,5.
