Pulmonary Artery Intimal Sarcoma Presenting as Acute Pulmonary Embolism - A Case Report

肺動脈肉瘤是非常罕見且高度惡性之腫瘤。大部份是起源於肺動脈的內膜細胞。肺動脈內膜肉瘤的臨床表現及放射線的特徵和急性肺栓塞非常相似，因此在早期，兩者非常難做鑑別診斷。在此，我們報告一個57 歲的男性病患，因為突發性咳嗽，胸悶，痰內有血絲等症狀，而來急診就醫。生化及血液檢查結果都正常。但是胸部X 光發現兩側肺部有多顆結節。電腦斷層顯示除了肺部結節外，肺動脈主幹有充盈缺損。懷疑是急性肺栓塞或是在肺動脈的轉移性癌症。電腦斷層導引切片的病理報告為不明來源的惡性肉瘤。病人接受肺動脈腫瘤切除及肺部結節楔型切除。最後病理診斷為肺動脈內膜肉瘤。之後病人接受化療，肺葉切除，及腦部轉移腫瘤的放射治療。但是病人情況持續惡化，在初步表現症狀後13 個月後過世。

關鍵字

肺動脈內膜肉瘤；急性肺栓塞

並列摘要

Pulmonary artery sarcomas are very rare and highly malignant tumors. Most are considered to arise from the pluripotent intimal cells of the pulmonary artery. The clinical symptoms/signs and radiological features of pulmonary artery intimal sarcoma are very similar to those of pulmonary thromboembolism, so it is very difficult to diagnose in the early stage. Here, we presented the case of a 57-year-old man who visited the hospital’s Emergency Service due to a sudden onset of cough with chest tightness and blood-tinged sputum. The laboratory data were within normal limits. However, chest X-ray showed multiple nodular lesions at bilateral lung fields and CT scan revealed bilateral lung nodules and multiple filling defects in the pulmonary trunk. Acute pulmonary embolism or metastatic tumors with tumor thrombi in the pulmonary arteries were suspected. CT-guided biopsy of the lung nodule revealed an undifferentiated sarcoma of uncertain origin. Complete excision of the pulmonary trunk tumor and wedge resection of the lung nodules were performed. The diagnosis was pulmonary artery intimal sarcoma with multiple lung extensions. Chemotherapy, additional lobectomy, and radiotherapy to the brain for the metastatic tumors were all tried, but the tumor progressed and the patient expired 13 months after the initial presentation.

並列關鍵字

pulmonary artery intimal sarcoma ； pulmonary thromboembolism

國際替代計量

Pulmonary Artery Intimal Sarcoma Presenting as Acute Pulmonary Embolism - A Case Report

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