肺淋巴管平滑肌瘤病临床病理观察

Pulmonary lymphangioleiomyomatosis:a clinicopathologic analystis

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.