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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 investigate the clinical and histopathological features of pulmonary lymphatic leiomyoma (PLAM). Methods 1 case of PLAM clinical data analysis, histological observation and immunohistochemistry, and review the relevant literature. Results Female patient, 30 years old. Chest tightness, chest pain 1 week, chest X-ray showed spontaneous pneumothorax. Histology showed hyperplastic smooth muscle-like cells around the lumen around the abnormal proliferation of lung parenchymal cystic degeneration. Immunohistochemistry: ɑ-SMA, ER, PR, desmin, HMB45 and β-catenin (+). Conclusion PLAM is a rare neoplastic disease with progressive chronic lung degeneration. According to clinical and histopathological features, PLAM can be diagnosed by immunohistochemical staining.