Surgical treatment of primary pulmonary sarcoma disseminated to both pulmonary arteries using composite pulmonary trunk and bifurcation of abdominal aorta allograft

Primary pulmonary artery sarcoma (PPAS) is a rare tumor originating from the intimal mesenchymal cells of the pulmonary artery with an incidence of 0.001–0.03% and is usually fatal [1]. The diagnosis is difficult and delayed in most cases. It usually presents in adults (almost equally in males and females), with median age of presentation of 50 years [2]. Newer imaging techniques could allow early diagnosis in patients with symptoms of pulmonary vascular obstruction [3]. Surgical resection improves clinical symptoms, offers the only chance of cure and is performed in many cardiothoracic clinics. The PPAS spreading to lobular branches of pulmonary artery (PA) represents an especially serious surgical challenge.
A 58-year-old female patient was referred to our unit in December 2016 complaining of dyspnea on moderate physical exertion and unproductive cough starting 2 months prior to admission. Transthoracic echocardiography revealed a mass formation of 62/24 mm in the pulmonary trunk, fused with the valvular leaflets. Third-degree tricuspid valve regurgitation was recorded as well. Aortic and mitral valves showed no significant change, with left ventricular ejection fraction (LVEF) of 62%, and right ventricular ejection fraction (RVEF) of 42%. Laboratory findings revealed no significant changes. Computed tomography angiography (CTA) showed a negative dynamic in comparison to CT data of a month earlier: the tumor spread from the bifurcation of the pulmonary trunk by 5 mm to the left branch of the pulmonary artery, to the bifurcation of the right branch of the PA and up to 10 mm to the valvular space of the right ventricular outflow tract (Fig. 1 A). Tumor emboli in the segmental branches of the PA were also seen. In the lung parenchyma, foci appeared to have increased in size: 4 mm in S6 on the right (+3 mm), 6 mm in S3 on the left (+1–2 mm), 7 mm in S6 on the left (newly formed). Positron emission tomography (PET) confirmed the presence of metabolic-active formation in the pulmonary truck spreading to both PA and secondary changes in S6 of the right and in S3 and S6 of the left lung. In this case changes in the lungs were assumed as secondary to primary sarcoma of the PA.
Surgery was performed on December 28, 2016 through median sternotomy. Before institution of cardiopulmonary bypass (CPB) and following a revision of the lungs, an atypical resection of the foci in S6 of the right lung and in S3 and S6 of the left lung was performed. Upon...


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