Surgical treatment of primary pulmonary artery sarcoma
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ORIGINAL ARTICLE
Surgical treatment of primary pulmonary artery sarcoma Yongxin Han1 · Yanan Zhen2 · Xiaopeng Liu2 · Xia Zheng2 · Jianbin Zhang2 · Zhenguo Zhai3 · Jun Duan4 · Yajun Zhang5 · Peng Liu1,2 Received: 20 July 2020 / Accepted: 27 August 2020 © The Author(s) 2020
Abstract Objective Primary 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. Methods From 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. Results All 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. Conclusions PAS 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. Keywords Pulmonary artery sarcoma · Pulmonary thromboendarterectomy · Postoperative chemotherapy
Introduction
Yongxin Han and Yanan Zhen have contributed to the work equally. * Peng Liu [email protected] 1
Peking University China-Japan Friendship School of Clinical Medicine, Beijing 100029, China
2
Department of Cardiovascular Surgery, China-Japan Friendship Hospital, Cherry Park East Street, Chaoyang District, Beijing 100029, China
3
Respiratory Department, China-Japan Friendship Hospital, Beijing, China
4
Surgical Intensive Care Unit, China-Japan Friendship Hospital, Beijing, China
5
Surgical Anesthesia Department, China-Japan Friendship Hospital, Beijing, China
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. Transthoracic and transesophageal echocardiography (TTE and TEE), computed tomography pulmonary angiography (CTPA), cardiac magnetic resonance imaging (MRI), endobronchial ultrasound transbronchial needle aspiration (EBUS-TBNA) and fluorodeoxyglucose positron
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