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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 chest and abdomen in patients with lymphangiomyomatosis (LAM). Materials and Methods Imaging features of thorax and abdomen in 12 patients with pathologically confirmed LAM were analyzed. Results The CT findings of chest CT showed that there were no wall or thin wall cysts with diameter of 0.5 ~ 2 cm in both lungs. Among them, 10 cysts were diffusely distributed, 1 was scattered and 1 had only small cysts. There were 7 patients with pneumothorax and pleural effusion, and 11 patients (92%) had abnormalities in the abdomen, including 4 cases of multiple retroperitoneal lymphangiomyoma (2 patients with massive peritoneal and pelvic effusion, 1 patient with ovarian Teratoma, 1 case with bilateral ovarian cyst), liver and kidney and pelvic multiple myeloma in 1 case, with renal angiomyolipoma in 1 case; in addition to 7 cases, 2 cases of uterine fibroids, multiple liver and kidney Angiomyolipoma with uterine fibroids, gallbladder polyps with uterine fibroids, multiple hepatic steatoma with uterine fibroids, liver cysts, ascites in 1 case. Conclusion LAM is a multi-system disease, and its chest and pelvic lesion imaging has certain characteristics.