Chan Edward Y, Ravi Vinod, Ali Areeba, Nguyen Duc T, Graviss Edward A, MacGillivray Thomas E, Reardon Michael J
Department of Surgery, Houston Methodist Hospital, Houston, Texas.
Department of Sarcoma Oncology, MD Anderson Cancer Center, Houston, Texas.
Semin Thorac Cardiovasc Surg. 2023 Spring;35(1):53-64. doi: 10.1053/j.semtcvs.2021.10.013. Epub 2021 Nov 4.
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，若不进行手术治疗，预后很差。它常与更常见的慢性肺栓塞相混淆，这可能会延迟正确诊断或导致手术效果欠佳。本研究的目的是评估在我们机构采用解剖切除方法处理肺动脉干和主肺动脉而非内膜剥脱术治疗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月在我们机构接受手术的原发性肺肉瘤病例，并随访至2021年1月3日。对于受累的肺根和主肺动脉，我们采用解剖切除和置换技术，仅在可能保留肺组织时，对第一动脉分支远端的病变采用内膜剥脱术。本研究的主要终点是从初始诊断时起的生存率以及从我们手术时起的生存率。次要终点是手术30天死亡率以及局部复发或转移性疾病的发生率。我们确定了20例连续的原发性肺肉瘤手术切除病例。手术时的中位年龄为52.5岁（四分位间距43.5 - 60.5岁）。20例中的16例（80%）需要使用同种异体肺移植进行完整的肺根切除和重建。所有切除均采用体外循环和心脏停搏。20例患者中的7例（35%）需要进行肺切除术。9例患者（45%）实现了切缘阴性（R0）切除，9例患者（45%）在最终病理检查中切缘显微镜下阳性（R1）。2例患者（10%）在切除边缘有肉眼可见的肿瘤（R2）。手术死亡率为2/20（10%）。从诊断起的中位生存期为2.8年（95%可信区间1.3 - 8.8），我们团队手术起的中位生存期为2.7年（95%可信区间0.8 - 5.9）。从首次初始诊断起1年、3年、5年和10年的生存率分别为85.0%、49.1%、49.1%和16.4%。我们团队手术起1年、3年、5年和10年的生存率分别为70%、48.8%、41.8%和8.4%。与该疾病的自然病程相比，采用解剖切除受累的肺根和主肺动脉、对第一分支动脉远端有病变时行肺切除术或内膜剥脱术的方法手术切除原发性肺动脉肉瘤，手术风险合理且有长期生存。
