Primary Pulmonary Artery Sarcoma Mimicking Pulmonary Thromboembolic Disease: A Case Report

類似肺動脈血栓栓塞性疾病的原發性肺動脈肉瘤：一個病例報告

陳志誠(Chih-Cheng Chen) ； 鄭之勛(Jih-Shuin Jerng)

《胸腔醫學》 33卷6期 (2018/12) Pp. 230-236

摘要

Primary pulmonary artery sarcoma is a rare disease entity, and patients might have the typical presentations of thromboembolic disease. We report a 50-year-old woman who presented with a 3-month history of dyspnea on exertion and chest tightness. Chest X-ray examination revealed a prominent right pulmonary trunk and a nodule at the left lower lung field; magnetic resonance imaging disclosed significant ＂filling defects＂ within bilateral pulmonary trunks. With the clinical diagnosis of pulmonary thromboembolism, she was treated with systemic anticoagulants, but the symptoms and radiographic findings progressed. Surgical biopsy of the pulmonary artery lesion revealed poorly differentiated pulmonary artery sarcoma. She died 1 year later despite treatment. Our case illustrates that pulmonary artery sarcoma should be included in the differential diagnosis of pulmonary thromboembolic disease when there is a lack of response to anticoagulation in patients with no evident risk factor for pulmonary thromboembolism, and in those with concomitant pulmonary nodules.

關鍵字

pulmonary artery sarcoma ； pulmonary thromboembolism ； pulmonary thromboembolic disease

並列摘要

原發性肺動脈肉瘤是很罕見的疾病，病人經常有像肺血栓栓塞性疾病的典型表現。我們報告了一位50歲女姓，她的表現是長達三個月期間的運動喘及胸悶。胸部X 光檢查發現右肺動脈幹變得很鼓且在左下肺葉有一顆結節。核磁共振掃描發現在兩側肺動脈幹有所謂“填充缺損”。臨床診斷為肺動脈血栓栓塞症，於是她開始使用抗凝血劑治療。追蹤時，其症狀及影像皆惡化。肺動脈的病灶經開刀後病理診斷為分化不良性的肺動脈肉瘤。然而即使經過開刀、電療及化療，病人仍在診斷的一年多後死亡。我們的案例闡明了：當病人沒有血栓栓塞症的危險因子，對於抗凝血劑治療沒有反應而且合併有肺結節者，肺動脈栓塞症以外的疾病像是肺動脈肉瘤一定要列入鑑別診斷。

並列關鍵字

肺動脈肉瘤 ； 肺動脈血栓栓塞症 ； 肺動脈血栓栓塞性疾病