Primary pulmonary artery sarcoma is a rare cardiac tumor with a dismal prognosis without surgical therapy. It is often confused with the more common chronic pulmonary emboli which may delay the appropriate diagnosis or lead to suboptimal surgery. The objective of this study was to evaluate the short and long-term survival and local recurrence rate of pulmonary artery sarcoma cases operated on at our institution using an anatomic resection approach for the pulmonary trunk and main pulmonary arteries rather than endarterectomy. We searched our prospectively collected cardiac tumor database for cases of primary pulmonary sarcoma operated at our institution between June 2000 and September 2018 and followed until January 3, 2021. We used an anatomic resection and replacement technique for involved pulmonary root and main pulmonary arteries with endarterectomy used only for disease distal to the first arterial branch when lung preservation was possible. The primary endpoints for our study were survival from the time of initial diagnosis and survival from the time of our surgery. Secondary endpoints were operative 30-day mortality and incidence of local recurrence or metastatic disease. We identified 20 consecutive cases of surgical resection of primary pulmonary sarcoma. The median age at surgery was 52.5 years (IQR 43.5–60.5). Complete pulmonary root resection and reconstruction using a pulmonary homograft were needed in 16/20 (80%) of cases. All resections employed cardiopulmonary bypass with cardioplegic arrest. A pneumonectomy was needed in 7/20 (35%) of patients. A negative margin (R0) resection was achieved in 9 patients (45%) and margins were microscopically positive (R1) on final pathology in 9 patients (45%). Two patients (10%) had gross tumor (R2) at the resection margin. Operative mortality was 2/20 (10%). Median survival was 2.8 years from diagnosis (95% CI 1.3–8.8) and 2.7 years from surgery by our team (95% CI 0.8–5.9). Survival from first initial diagnosis at 1, 3, 5, and 10 years was 85.0%, 49.1%, 49.1%, and 16.4%. Survival from our surgery by our team at 1, 3, 5, and 10 years was 70%, 48.8%, 41.8%, and 8.4%. Surgical resection of primary pulmonary artery sarcoma with an approach utilizing an anatomic resection of the pulmonary root and main pulmonary arteries when involved and pneumonectomy or endarterectomy when there is disease distal to the first branch artery can be done with a reasonable operative risk and long-term survival when compared to the natural history of the disease.

原发性肺动脉肉瘤是一种罕见的心脏肿瘤，如果不进行手术治疗，预后很差。它经常与更常见的慢性肺栓塞混淆，后者可能会延迟适当的诊断或导致次优手术。本研究的目的是评估在我们机构使用肺干和主要肺动脉解剖切除方法而不是动脉内膜切除术手术的肺动脉肉瘤病例的短期和长期生存率以及局部复发率。我们在我们前瞻性收集的心脏肿瘤数据库中搜索了2000 年 6 月至 2018 年 9 月期间在我们机构手术并随访至 2021 年 1 月 3 日的原发性肺肉瘤病例。我们对受累的肺根和主肺动脉采用解剖切除和置换技术，在可以保留肺的情况下，动脉内膜切除术仅用于第一动脉分支远端的疾病。我们研究的主要终点是从初步诊断时起的存活率和从我们手术时起的存活率。次要终点是手术 30 天死亡率和局部复发或转移性疾病的发生率。我们确定了 20 例原发性肺肉瘤手术切除的连续病例。接受手术的中位年龄为 52.5 岁 (IQR 43.5–60.5)。16/20 (80%) 的病例需要使用肺同种移植物进行完全肺根切除和重建。所有切除术均采用心肺旁路心脏停搏。A7/20 (35%) 的患者需要进行全肺切除术。9 名患者 (45%) 实现了切缘阴性 (R0) 切除，9 名患者 (45%) 的最终病理结果为显微镜下阳性 (R1)。两名患者 (10%) 在切除边缘有肉眼可见的肿瘤 (R2)。手术死亡率为 2/20 (10%)。诊断后的中位生存期为 2.8 年 (95% CI 1.3–8.8)，我们团队进行手术后的中位生存期为 2.7 年 (95% CI 0.8–5.9)。生存从第一的1 年、3 年、5 年和 10 年的初诊率分别为 85.0%、49.1%、49.1% 和 16.4%。生存从我们的我们团队在 1、3、5 和 10 年的手术率为 70%、48.8%、41.8% 和 8.4%。原发性肺动脉肉瘤的手术切除，当累及肺根和主肺动脉时，采用解剖切除的方法；当第一支动脉远端有疾病时，可进行全肺切除术或动脉内膜切除术，手术风险合理且长期与疾病的自然史相比，存活率更高。