Clinical and pathological features and imaging manifestations of pulmonary lymphangioleiomyomatosis
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摘要: 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.
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