McLain Julian H, Khadarian Kevork, Shojaie Layla, Lubman Richard, Chang Ching-Fei, Lindgren Brett, Shao Ling
Department of Medicine, Keck School of Medicine, University of Southern California, Los Angeles, CA.
ACG Case Rep J. 2021 Mar 3;8(3):e00517. doi: 10.14309/crj.0000000000000517. eCollection 2021 Mar.
A 35-year-old woman presented to the hospital with a 4-week history of large-volume chylous ascites refractory to paracentesis and new-onset dyspnea. Thoracic computed tomography revealed diffuse pulmonary cystic lesions with pleural effusions, and abdominal computed tomography showed ascites with large bilateral retroperitoneal masses displaying positron emission tomography avidity. Biopsy of the masses demonstrated lymphatic invasion by a perivascular epithelioid cell neoplasm, a smooth muscle tumor. The patient was diagnosed as having the sporadic form of lymphangioleiomyomatosis and was treated with the mammalian target of rapamycin pathway inhibitor sirolumus with clinical improvement.
一名35岁女性因大量乳糜性腹水4周，经腹腔穿刺治疗无效且出现新发呼吸困难而入院。胸部计算机断层扫描显示弥漫性肺囊性病变伴胸腔积液，腹部计算机断层扫描显示腹水伴双侧巨大腹膜后肿块，正电子发射断层扫描显示肿块有放射性摄取。肿块活检显示血管周上皮样细胞瘤（一种平滑肌肿瘤）侵犯淋巴管。该患者被诊断为散发型淋巴管平滑肌瘤病，并接受雷帕霉素靶蛋白通路抑制剂西罗莫司治疗，临床症状改善。
