Mahajan Nandita N, Cicek Sertac M, McGregor Christopher G A, Boland Jennifer M, Morris Jonathan M, Okuno Scott H, Robinson Steven I, White Darin B, Yi Eunhee S, Blackmon Shanda H
12346 Division of General Thoracic Surgery, Mayo Clinic, Rochester, MN, USA.
536580 Division of Cardiovascular Surgery, Mayo Clinic, Rochester, MN, USA.
Innovations (Phila). 2021 Jan-Feb;16(1):94-100. doi: 10.1177/1556984520960716. Epub 2020 Oct 20.
There are limited data regarding the surgical management of primary pulmonary artery sarcomas (PPAS) because of their rarity and complicated diagnostic history. The objective of this study was to analyze our institution's long-term surgical management outcomes for PPAS in the absence of a care pathway. From May 1997 to June 2013, 8 patients (mean age 60.6 ± 11.8 years; range, 40-73 years; 5 women and 3 men) underwent surgical intervention for PPAS at our institution. The most common computed tomography finding was a luminal filling defect obstructing the pulmonary artery (PA), without evidence of extraluminal extension. Three patients underwent debulking/pulmonary endarterectomy alone and 5 patients underwent a more radical resection with PA patch angioplasty, PA resection and reconstruction, pulmonary valve replacement, and unilateral pneumonectomy. The mean postoperative survival in this series was 3.8 ± 3.6 years (range, 1-11.9 years), with 2 radical surgical resection patients alive at 4.9 and 11.9 years, respectively. For those patients with incomplete resection, 3-dimensional (3D) models were created to demonstrate the advantage of a preoperative guide for a more complete resection and what it would entail. Six patients had local recurrences with mean disease-free interval of 14 ± 10.9 months (range, 2 months-2.5 years), and 2 patients with re-resections had an overall postoperative survival of 2.8 and 11.9 years, respectively. In our small cohort of PPAS, patients treated with radical surgical resection had better survival. The small number of PPAS cases in this series makes proving this association unlikely but warrants consideration.
由于原发性肺动脉肉瘤（PPAS）罕见且诊断史复杂，关于其外科治疗的数据有限。本研究的目的是分析在没有护理路径的情况下，我们机构对PPAS的长期外科治疗结果。1997年5月至2013年6月，8例患者（平均年龄60.6±11.8岁；范围40 - 73岁；5例女性和3例男性）在我们机构接受了PPAS的外科干预。计算机断层扫描最常见的表现是管腔充盈缺损阻塞肺动脉（PA），无腔外扩展证据。3例患者仅接受了减瘤/肺动脉内膜切除术，5例患者接受了更彻底的切除术，包括PA补片血管成形术、PA切除和重建、肺动脉瓣置换及单侧肺切除术。该系列患者的平均术后生存期为3.8±3.6年（范围1 - 11.9年），2例接受根治性手术切除的患者分别在4.9年和11.9年存活。对于那些切除不完全的患者，创建了三维（3D）模型以展示术前指导进行更完全切除的优势及其所需条件。6例患者出现局部复发，平均无病间隔为14±10.9个月（范围2个月 - 2.5年），2例再次手术切除的患者术后总生存期分别为2.8年和11.9年。在我们这个小样本的PPAS队列中，接受根治性手术切除的患者生存期更好。本系列中PPAS病例数量较少，虽不太可能证实这种关联，但值得考虑。
