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

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.