淋巴管肌瘤病的临床、影像学与病理特点

目的和方法 探讨淋巴管肌瘤病（ＬＡＭ）的临床表现、影像学特点、病理改变及治疗转归。结果 ２例均为育龄妇女,其特点为进行性呼吸困难,反复发作气胸、乳糜胸、咯血、腹水和腹痛;肺部高分辨率ＣＴ典型表现为;双肺广泛薄壁囊状影;基本病理特征为：淋巴系统、肺淋巴管、小血管、呼吸性细支气管及其周围平滑肌细胞进行性病态增生,引起局部管腔的狭窄或阻塞,以致出现一系列临床症状。结论 病理学上发现淋巴系统、小血管 、肺

A Clinical , Radiographic , Pathological and Therapeutic Study of 2 Cases Lymphangiomyomatosis

Objective and Methods The study was to investigate the clinical,radiographic and pathological features of Lymphangiomyomatosis (LAM). Results The 2 patients who were all females of childbearing age had an onset with progressive dyspnea,recurrent pneumothorax,chylous effusion and hemoptysis,thoracic or abdominal pain. High limited computer tomagraphy showed numerous cysts in both lungs in all the 2 patients. Light microscopic examination showed that abnormal smooth muscle proliferation scattered throughout the lungs,obstructing the small aieways,venules and lymphatics. The definite dignosis of a retroperitoneal tumor was LAM. Conclusion The pathological findings with proliferation of smooth muscle cells within the lymphatics,mediastinal and retro|peritoneal lymph nodes and in the lungs might be the crucial procedure for making a definite diagnosis of LAM. [