Chan S H, Tsai L M, Tsai W C, Li Y H, Chen J H, Luo C Y
Department of Medicine, National Cheng Kung University Hospital, Tainan, Taiwan.
J Formos Med Assoc. 1999 Aug;98(8):578-81.
Pulmonary artery leiomyosarcoma is a rare but highly lethal disease, and can be mistaken for pulmonary thromboembolism. We report a case of pulmonary artery leiomyosarcoma managed with surgical resection, chemotherapy, and radiotherapy. A 57-year-old woman was admitted with complaints of aggravated dyspnea. She was initially treated with oxygen therapy and heparinization for a suspected pulmonary embolism. Echocardiography revealed a dilated right atrium and ventricle and severe tricuspid regurgitation, with an estimated systolic right ventricular pressure of 95 mm Hg; a shadow of a mass in the main pulmonary artery was also noted. Right ventriculography revealed a filling defect, and to-and-fro motion of the mass in the main pulmonary artery. The left pulmonary artery was almost totally occluded by the mass. The patient's condition improved dramatically after palliative excision of the mass and patch reconstruction of the outflow tract of the right ventricle with a bicuspid xenograft. Pathologic examination of the mass revealed leiomyosarcoma. Chemotherapy and radiotherapy were subsequently administered and follow-up imaging studies 3 months postoperatively revealed no recurrence of the tumor. The patient remains well, more than 1 year after treatment. This report emphasises that pulmonary artery sarcoma should be considered in the differential diagnosis in cases of suspected pulmonary thromboembolism.
肺动脉平滑肌肉瘤是一种罕见但致死率很高的疾病，可能会被误诊为肺血栓栓塞症。我们报告一例接受手术切除、化疗和放疗的肺动脉平滑肌肉瘤病例。一名57岁女性因呼吸困难加重入院。她最初因疑似肺栓塞接受了氧疗和肝素化治疗。超声心动图显示右心房和右心室扩大，严重三尖瓣反流，估计右心室收缩压为95mmHg；还注意到主肺动脉有一个肿块阴影。右心室造影显示有充盈缺损，主肺动脉内肿块有往返运动。左肺动脉几乎完全被肿块阻塞。在对肿块进行姑息性切除并用双叶异种移植物对右心室流出道进行补片重建后，患者的病情显著改善。肿块的病理检查显示为平滑肌肉瘤。随后进行了化疗和放疗，术后3个月的随访影像学检查未发现肿瘤复发。治疗1年多后，患者情况良好。本报告强调，在疑似肺血栓栓塞症的病例中，鉴别诊断时应考虑肺动脉肉瘤。
