肺淋巴管平滑肌瘤病的临床病理学分析

Clinicopathological features of pulmonary lymphangioleiomyomatosis

目的 探讨肺淋巴管平滑肌瘤病(PLAM)的临床特点、影像学及病理学特征。方法 对61例PLAM进行临床资料、组织学形态、影像学及免疫组化分析,并复习相关文献。结果 患者均为女性,年龄21~59岁,平均年龄(34.70±12.37)岁。多以活动后气促、进行性呼吸困难、反复自发性气胸、咳嗽等入院。影像学为两肺多发圆形薄壁囊状影,12例伴有气胸。肺功能检查多表现为阻塞性通气功能障碍和弥散功能障碍。镜下肺组织内见增生的短梭形平滑肌样或上皮样LAM细胞呈小簇状或结节状分布于囊壁或者围绕血管、淋巴管和细支气管壁上。免疫组化:LAM细胞HMB45、SMA、PR及ER均不同程度地表达。结论 肺淋巴管平滑肌瘤病是一种肺部罕见局部破坏的间叶性恶性肿瘤,归属于血管周上皮细胞肿瘤家族,好发生于育龄期女性,影像学检查双肺常呈圆形的薄壁囊状影,明确诊断需要依靠病理学检查。

Objective To investigate the clinicophathological and imaging features of pulmonary lymphangiomyomatosis(PLAM). Methods The clinical data, histological morphology, imaging and immunohistochemistry of 61 cases of PLAM were analyzed, and the relevant literature was reviewed. Results All patients were female, aged from 21 to 59 years, with an average age of 34.70±12.37 years. Most of them were hospitalized with shortness of breath after activities, progressive dyspnea, repeated spontaneous pneumothorax, cough and so on. Imaging showed multiple round thin-walled cysts in both lungs, with pneumothorax in 12 cases. Pulmonary function examination showed obstructive ventilation dysfunction and diffusion dysfunction. Microscopically, proliferative short spindle smooth muscle-like or epithelioid LAM cells were seen in the lung tissue, distributed in small clusters or nodules on the cystic wall or around the walls of blood vessels, lymphatic vessels and bronchioles. Immunohistochemically, HMB45, SMA, PR and ER were expressed in LAM cells. Conclusion PLAM is a malignant mesenchymal neoplasm with local destruction, belonging to the family of perivascular epithelial cell tumors, which usually occurs in women of childbearing age. The imaging examination of both lungs often shows round thin-walled cystic shadow. The definite diagnosis relies on pathological examination.