Zhang Zhaohua, Zhen Yanan, Liu Jingwen, Liu Xiaopeng, Yang Liang, Xu Mingyuan, Wen Jianyan, Liu Peng
Peking University China-Japan Friendship School of Clinical Medicine, Beijing, China.
Department of Cardiovascular Surgery, China-Japan Friendship Hospital, Beijing, China.
Front Cardiovasc Med. 2024 Jul 2;11:1302372. doi: 10.3389/fcvm.2024.1302372. eCollection 2024.
Pulmonary artery sarcoma (PAS) is an exceedingly rare and insufficiently investigated disease, leading to uncertain in its optimal management. This study aims to present our institutional experience and the outcomes of pulmonary endarterectomy for PAS.
We gathered clinical characteristics, intraoperative data, postoperative outcomes, and prognosis information from PAS patients who underwent surgical treatment at our institution between December 2016 and September 2023.
A total of 20 patients with PAS underwent pulmonary endarterectomy. The median age of the patients was 52 (IQR 45, 57) years, with 12 patients (60%) being female. Intimal sarcoma was confirmed in 19 patients, while the remaining one was diagnosed with large cell neuroendocrine carcinoma. The perioperative mortality rate was three cases (15%). Follow-up was conducted for a median duration of 14 months (range: 1-61). During the follow-up period, 11 patients experienced recurrence or metastasis, and 5 patients succumbed to the disease. The estimated cumulative survival rates at 1 and 2 years for all 20 patients were 66.4% and 55.3%, respectively.
Pulmonary endarterectomy emerges as a palliative but effective approach for managing PAS, particularly when complemented with postoperative therapies such as chemotherapy and targeted therapy, which collectively contribute to achieving favorable long-term survival outcomes.
肺动脉肉瘤（PAS）是一种极为罕见且研究不足的疾病，其最佳治疗方案尚不确定。本研究旨在介绍我们机构的经验以及肺动脉内膜剥脱术治疗PAS的结果。
我们收集了2016年12月至2023年9月期间在我们机构接受手术治疗的PAS患者的临床特征、术中数据、术后结果和预后信息。
共有20例PAS患者接受了肺动脉内膜剥脱术。患者的中位年龄为52岁（四分位间距45，57），其中12例（60%）为女性。19例患者确诊为内膜肉瘤，其余1例诊断为大细胞神经内分泌癌。围手术期死亡率为3例（15%）。中位随访时间为14个月（范围：1 - 61个月）。在随访期间，11例患者出现复发或转移，5例患者死于该疾病。所有20例患者1年和2年的估计累积生存率分别为66.4%和55.3%。
肺动脉内膜剥脱术是一种姑息但有效的PAS治疗方法，特别是在辅以化疗和靶向治疗等术后治疗时，这些治疗共同有助于实现良好的长期生存结果。
