肺淋巴管平滑肌瘤病的临床病理特征及影像学表现

目的肺淋巴管平滑肌瘤病（PLAM）的临床病理及影像学特征。方法观察2例肺淋巴管平滑肌瘤患者的临床特点、影像学表现、病理特征。结果患者肺组织特征性改变为两肺弥漫均匀分布的薄壁囊性气腔；肺活检病理示：沿肺淋巴管、小气道、小血管的管壁及其周围的平滑肌细胞弥漫性异常增生，免疫组织化学染色结果显示HMB45、SMA均阳性，ER、PR均阴性。结论PLAM临床罕见，是肺慢性进行性恶化的肿瘤性疾病，本病的确诊需要肺组织病理学检查。

Clinical and pathological features and imaging manifestations of pulmonary lymphangioleiomyomatosis

Objective To investigate the clinical and pathological features and imaging mani- festations of pulmonary lymphangioleiomyomatosis （PLAM）. Methods Two PLAM patients were ob- served in terms of clinical features, imaging manifestations and pathological features. Results The clinical manifestations of PLAM were progressive shortness of breath （dyspnea） , recurrent spontaneous pneumothorax, chylothorax and haemopthsis. PLAM carried characteristic high-resolution CT （HRCT） sings, which showed multiple thin-walled cysts distributed evenly throughout the bilateral lung fields. Pathologic examination of lung biopsy specimens showed abnormal, perivascular proliferation of smooth muscle in and around the small airway, lymphatic and small blood vessels. Immunophenotypic findings revealed positive staining for HMB-45, SMA, but negative for ER and PR. Conclusion PLAM is a rare neoplastic proliferative disease in the lung. Pathologic examination of lung tissue biopsy is re- quired for confirmation of PLAM diagnosis.