PDF(Pubmed)
Abstract：
UNASSIGNED: 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.
UNASSIGNED: 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.
UNASSIGNED: 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.
UNASSIGNED: 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.
UNASSIGNED: 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.
UNASSIGNED: 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.
UNASSIGNED: 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.
摘要：
■肺动脉肉瘤（PAS）是一种极为罕见且研究不足的疾病，导致其优化管理的不确定性。这项研究旨在介绍我们的机构经验和PAS肺内膜切除术的结果。
■我们收集了临床特征，术中数据,术后结果,以及2016年12月至2023年9月期间在我们机构接受手术治疗的PAS患者的预后信息.
■共有20例PAS患者接受了肺内膜切除术。患者的中位年龄为52岁(IQR45,57),12名患者（60%）为女性。在19例患者中证实了内膜肉瘤，其余1例被诊断为大细胞神经内分泌癌。围手术期死亡率为3例(15%)。随访的中位时间为14个月（范围：1-61）。在后续期间,11例患者复发或转移，5名患者死于该病。所有20例患者的1年和2年累积生存率分别为66.4%和55.3%。分别。
■肺动脉内膜切除术是治疗PAS的一种姑息但有效的方法，特别是当辅以术后治疗如化疗和靶向治疗时，它们共同有助于实现有利的长期生存结果。
■我们收集了临床特征，术中数据,术后结果,以及2016年12月至2023年9月期间在我们机构接受手术治疗的PAS患者的预后信息.
■共有20例PAS患者接受了肺内膜切除术。患者的中位年龄为52岁(IQR45,57),12名患者（60%）为女性。在19例患者中证实了内膜肉瘤，其余1例被诊断为大细胞神经内分泌癌。围手术期死亡率为3例(15%)。随访的中位时间为14个月（范围：1-61）。在后续期间,11例患者复发或转移，5名患者死于该病。所有20例患者的1年和2年累积生存率分别为66.4%和55.3%。分别。
■肺动脉内膜切除术是治疗PAS的一种姑息但有效的方法，特别是当辅以术后治疗如化疗和靶向治疗时，它们共同有助于实现有利的长期生存结果。
