肺淋巴管平滑肌瘤病2例的临床病理观察及文献复习

Pulmonary lymphangioleiomyomatosis:a report of 2 cases and literature review

目的探讨肺淋巴管平滑肌瘤病(pulmonary lymphangioleiomyomatosis,PLAM)的临床病理学特点、免疫表型、特殊染色特征及鉴别诊断。方法应用HE、免疫组织化学和特殊染色对我院诊断的2例PLAM进行临床病理分析,并复习相关文献,探讨其临床病理学特征及预后。结果2例患者均为女性,CT均提示双肺弥漫性病变,镜下见沿肺泡腔弥漫分布的病变组织由增生的平滑肌样细胞、上皮细胞及淋巴管和血管组成,其中一例另可见由慢性肉芽肿性炎和干酪样坏死构成的结核病灶。免疫组化显示SMA、HMB45、MelanA均阳性,同时伴随D2-40阳性的淋巴管或血管增生。结论PLAM为临床罕见的肺间质性病变,易漏诊误诊,需结合临床进行诊断。

Objective To investigate the clinicopathological features,immunophenotype,special staining features and differential diagnosis of pulmonary lymphangioleiomyomatosis(PLAM).Methods HE,immunohistochemistry and special staining were performed to analyze the clinicopathological features and prognosis of 2 cases of PLAM diagnosed in our hospital with relative literature being reviewed.Results The two patients were female.CT showed diffuse lesions in both lungs.Microscopically,the lesions were diffusely distributed along the alveolar cavity,and were composed of proliferative smooth muscle like cells,epithelial cells,lymphatic vessels and blood vessels.In one case,tuberculous lesions composed of chronic granulomatous inflammation and caseous necrosis were observed.Immunohistochemistry showed that SMA,HMB45 and Melania were positive,accompanied by D2-40-positive lymphangiosis or vascular proliferation.Conclusion PLAM is a rare pulmonary interstitial disease,which is easy to be missed and misdiagnosed.The diagnosis should be combined with clinical manifestations.