Abstract
ObjectivePrimary pulmonary artery sarcoma (PAS) is a rare tumor that originates from the intimal layer of the pulmonary artery or pulmonary valve and has a poor prognosis. The standard treatment for this devastating disease remains unclear. This study aimed to summarize the current standard treatments for PAS.MethodsFrom September 2015 to January 2020, six patients were diagnosed with PAS and underwent pulmonary endarterectomy (PEA) at our department. Their medical records were retrospectively reviewed to analyze the clinical characteristics, histopathological features, and postoperative outcomes. Fourteen articles, each reporting at least 6 cases, identified 201 patients diagnosed with PAS, and 158 patients had detailed treatments and follow-up data.ResultsAll of the patients who successfully underwent PEA were alive at follow-up, with a mean survival duration of 11.6 months (7–28 months), and one patient developed recurrence in the right upper lobe lung. Two patients received postoperative chemotherapy. In one patient, the tumor invaded the pulmonary valve.ConclusionsPAS resection combined with PEA via the aid of cardiopulmonary bypass and deep hypothermic circulatory arrest could achieve maximal tumor resection in patients without metastatic lesions. An individualized surgery strategy relies on a precise preoperative imaging examination. Moreover, postoperative adjuvant therapy could yield improved survival outcomes.
Highlights
Pulmonary hypertension (PH) is defined as a mean pulmonary artery pressure ≥ 20 mmHg at rest as assessed by right heart catheterization [1], and pulmonary artery sarcoma (PAS) along with chronic thromboembolic pulmonary hypertension (CTEPH) is categorized as Group 4 PH because of the similar hemodynamic index [2] and a pulmonary artery wedge pressure (PAWP) ≤ 15 mmHg
We report our experience and review the published cases to increase the understanding of PAS and to improve treatment modalities
From September 2015 to June 2020, 63 patients were diagnosed with PH and transferred to our department for surgical treatment, including 57 with CTEPH and 6 with PAS
Summary
Pulmonary hypertension (PH) is defined as a mean pulmonary artery pressure (mPAP) ≥ 20 mmHg at rest as assessed by right heart catheterization [1], and pulmonary artery sarcoma (PAS) along with chronic thromboembolic pulmonary hypertension (CTEPH) is categorized as Group 4 PH because of the similar hemodynamic index [2] and a pulmonary artery wedge pressure (PAWP) ≤ 15 mmHg. The prognosis of PAS is poor, and the median survival time without surgical resection was reported to be 1.5 months [3], while surgical resection of the tumor could lengthen the survival time to 8–36 months [7]. The. General Thoracic and Cardiovascular Surgery (2021) 69:638–645 main PAS treatment strategy is still unclear because of the limited number of reported cases in the literature. We report our experience and review the published cases to increase the understanding of PAS and to improve treatment modalities
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