Primary pulmonary artery sarcoma treated with simultaneous pulmonary valve replacement, artificial vessel...

Primary pulmonary artery sarcoma progresses extremely rapidly and has a poor prognosis. This condition is managed with surgical resection and multimodality therapy. However, standardized treatment is not available.

A 54-year-old woman was brought to the emergency department because of chest pain and worsening dyspnea that had developed in the past month. Contrast-enhanced computed tomography scan revealed severe stenosis extending from the pulmonary artery trunk to the right pulmonary artery due to an embolic substance. Because primary pulmonary artery sarcoma was suspected, emergency surgery was performed to improve the patient's symptoms. In addition to maximal tumor resection, pulmonary artery valve replacement, artificial vessel replacement, and right total pneumonectomy were performed. Based on the assessment performed using the specimen collected perioperatively, a pathological diagnosis of angiosarcoma of the right pulmonary artery was made. The patient was discharged on postoperative day 17 with a good postoperative course. However, because of dyspnea, she was readmitted to the hospital on day 85. Tumor recurrence was noted, and chemotherapy was initiated. The patient developed cardiac failure and died on postoperative day 119. A pathological postmortem examination was performed. Metastatic lesions were found in the pericardial sac, left lung, right chest wall and pleura, and mediastinum.

In the present case, postoperative recurrence was observed despite maximal resection of the surrounding tissues with tumor invasion and simultaneous reconstruction. Chemotherapy was initiated but was ineffective. Gene panel testing can help identify novel treatment options for patients with neoplastic diseases without standardized treatment. In addition, preparations should be made before surgery.