摘要
目的探讨肺淋巴管平滑肌瘤病(PLAM)的临床及病理组织学特征。方法对1例PLAM进行临床资料分析、组织形态学观察及免疫组化检测,并复习相关文献报道。结果患者女性,30岁。胸闷、胸痛1周,胸片示右侧自发性血气胸。组织学显示增生的平滑肌样细胞围绕管腔周围异常增生,肺实质囊性变。免疫组化:ɑ-SMA、ER、PR、desmin、HMB45和β-catenin(+)。结论 PLAM是罕见的肺慢性进行性恶化的肿瘤性疾病,依据临床和病理组织学特点,结合免疫组化染色可明确诊断。
Objective To discuss the clinical and pathological features of pulmonary lymphangioleiomyomatosis （PLAM）. Methods By using histological and immuuohistochemical （IHC） methods, the clinicopathological features were analyzed in I case of PLAM. Results Female patient of 30 years old, presented with chest tightness for a week. The chest-X-ray showed spoentanecus hemopneumothorx of the right side. The pathological features of PLAM were abnormal smooth muscle-like cells proliferation surrounding airways, lymphatics and blood vessels, which led to the cystic destruction of the lung parenchyma. IHC showed that characteristic smooth muscle-like cells were positive for a-SMA, ER, PR, desmin, 13-catenin and HMB45. Conclusion Lymphangioleiomyomatosis is an uncommon and progressive pulmonary tumor. The diagnosis can be made by evaluation of the clinical characteristics, histological and immunohistochemical features.
出处
《诊断病理学杂志》
CSCD
北大核心
2013年第2期78-80,84,共4页
Chinese Journal of Diagnostic Pathology
关键词
肺淋巴管平滑肌瘤病
临床病理学
免疫组化
诊断
Pulmonary lymphangioleiomyomatosis
Clinical pathology
Immunohistochemistry
Diagnosis
