Surgical Treatment of Primary Intimal Pulmonary Sarcoma

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  • 肺動脈原発血管内膜肉腫に対する外科治療の経験

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Primary pulmonary sarcoma is a very rare tumor. The prognosis of primary pulmonary sarcoma is extremely poor and it is reported as 1.5 months without treatment. Here we report our experience of surgical treatment of primary pulmonary sarcoma. A 62-year old woman with a diagnosis of pulmonary sarcoma by chest CT scan was seen in the university of Tsukuba Hospital because of cough and hemosputum. A large tumor was arising from the left pulmonary artery and extending into the right pulmonary artery and the main pulmonary trunk. An emergent surgery was planned to eliminate the risk of sudden death. Upon induction of anesthesia, her circulatory condition was rapidly collapsed and her heart was arrested. The emergent sternotomy was performed and she was placed on extracorporeal bypass. The main pulmonary artery and the right pulmonary artery were opened. The tumor stacking in the right pulmonary artery was removed and resected with the main pulmonary artery wall. The defect was repaired with a bovine pericardial patch. After extracorporeal bypass termination, the left pneumonectomy was completed. Pathologically, the tumor was diagnosed as an intimal sarcoma of the pulmonary artery. The surgical margin was negative. An adjuvant therapy was not admitted. At 36 months after surgery, she is doing well without any evidence of recurrence. In conclusion, intimal pulmonary sarcoma was successfully treated with the complete resection of the tumor by the resection of the main pulmonary artery and the left pneumonectomy. Aggressive surgical resection would be the most effective treatment to prolong survival for this malignant disease.

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