淋巴管肌瘤病的胸腹盆部影像学表现

Imaging of Lymphangiomyomatosis in Thorax and Abdominopelvic

目的探讨淋巴管肌瘤病(LAM)的胸部及腹盆部影像学表现。资料与方法分析12例经病理证实的LAM患者胸部及腹盆部影像学表现。结果胸部CT表现为双肺多发直径约0.5～2 cm的无壁或薄壁的小囊腔,其中10例囊状影弥漫性分布,1例散在分布,1例仅有数个小囊腔;伴有气胸、胸腔积液7例;11例(92%)患者腹盆部出现异常,其中腹膜后多发淋巴管肌瘤4例(2例伴有腹腔及盆腔大量积液,其中1例伴有卵巢畸胎瘤、1例伴有双侧卵巢囊肿)、合并肝肾及盆腔多发血管肌脂瘤1例、合并肾血管肌脂瘤1例;此外7例中,子宫肌瘤2例,肝肾多发血管肌脂瘤伴子宫肌瘤、胆囊息肉伴子宫肌瘤、肝多发血管肌脂瘤伴子宫肌瘤、肝脏囊肿、腹腔积液各1例。结论 LAM是一种多系统的疾病,其胸部及腹盆部病变影像学有一定特征性。

Objective To investigate the imaging findings of lymphangiomyomatosis（LAM） in thorax and abdominopelvic.Materials and Methods Imaging data of 12 cases with thorax and abdominopelvic LAM confirmed by pathology were analyzed retrospectively.Results CT findings of LAM included multiple small pulmonary cysts,which were about 5 to 20 millimeters in diameter with imperceptible-wall or thin-wall.Among these 12 cases,10 cases with pulmonary cysts were diffusely distributed;one case was scattered and one had a few cysts.7 cases were complicated with pneumothorax and pleural effusion.11 patients（92%） had abnormal abdominopelvic findings,retroperitoneal lymphangioleiomyoma in 4 cases（Two cases with massive ascites,and one was complicated with ovarian teratoma and uterine leiomyoma,the other had ovarian cysts）,complicated with hepatic and renal angiomyolipoma in 1 case,complicated with renal angiomyolipoma in 1 case,and in the other 7 cases,uterine leiomyoma in 2 cases,hepatic and renal multiple angiomyolipomas with uterine leiomyoma,gallbladder polyp with uterine leiomyoma,multiple hepatic angiomyolipomas with uterine leiomyoma,hepatic cysts,ascites in 1 case respectively.Conclusion LAM is a multisystem disease,which has typical imaging findings of thorax and abdominopelvic.