肺淋巴管肌瘤病3例报告并文献复习

Pulmonary lymphangioleiomyomatosis:Report of three cases and review of literature

目的探讨肺淋巴管瘤病的临床及病理组织学特征。方法应用光镜结合免疫组织化学标记对3例肺淋巴管瘤病进行临床病理分析,结合相关文献展开讨论。结果 3例均发生于女性。病理学检查显示不同成熟度平滑肌细胞在细支气管壁、肺泡壁、淋巴管壁和血管壁周围增生,肺实质呈囊性变,增生的平滑肌细胞免疫组织化学3例均显示平滑肌肌动蛋白SMA、黑色素瘤标记HMB45、雌激素受体、孕激素受体阳性。结论肺淋巴管瘤病是一种少见而独特的良性肿瘤。病理检查可确定肺淋巴管平滑肌瘤病的诊断。

Objective To study the clinical and pathological features of pulmonary lymphangioleiomyomatosis（PLAM）.Methods Three cases of PLAM were studied by microscopy and immunohistochemistry,with review of related literature.Results Three cases were all female.Pathological features showed abnormal smooth cells（LAM cells） line the airways,lymphatics and blood vessels leading to airflow obstruction and replacement of the lung parenchyma by cysts LAM cells were positive for HMB45,SMA,ER,PR.Conclusion PLAM is a rare distinct benign entity.Pathologic examination of lung tissue biopsy is required for confirmation of PLAM diagnosis.