Department of Surgery, Houston, Tex.
Houston Methodist Research Institute, Houston, Tex.
J Thorac Cardiovasc Surg. 2022 Jul;164(1):158-166.e1. doi: 10.1016/j.jtcvs.2020.09.100. Epub 2020 Oct 3.
Cardiac paraganglioma is a rare tumor that most surgeons have limited experience treating. The objective of this study is to examine the management and outcomes for cardiac paraganglioma treatment when cared for by a multidisciplinary cardiac tumor team.
We reviewed our institutionally approved cardiac tumor database from March 2004 to June 2020 for cardiac paraganglioma. These prospectively collected data were retrospectively reviewed. Patient characteristics were presented for individual patients and as summary statistics. Demographic and clinical data were also reported as median and interquartile range for continuous variables and frequencies and proportions for categoric variables. Kaplan-Meier curves were used to depict the patient survival from surgery.
There were 21 cases of primary cardiac paraganglioma, 19 of whom had surgical resection with 3 refusing offered surgery. Of 19 resected tumors, 13 originated from the left atrium and 6 originated from the roots of the pulmonary artery and the aorta. Complex procedures were required, including aortic and pulmonary root replacement and 8 autotransplants. All tumors had complete gross resection with no identifiable disease left behind, but 4 of these had microscopically positive margins. None of the patients had local recurrence of disease. There was 1 case of metastatic paraganglioma with death at 4 years postsurgery. Operative mortality was 10.6%. Survival from surgery was 88.2%, 71.8%, and 71.8% and 1, 5, and 10 years, respectively.
Cardiac paraganglioma presents a surgical challenge. Mortality and long-term survival after surgical resection are acceptable but may require complex resection and reconstruction.
心脏副神经节瘤是一种罕见的肿瘤，大多数外科医生在治疗方面经验有限。本研究的目的是检查多学科心脏肿瘤团队治疗心脏副神经节瘤时的治疗管理和结果。
我们回顾了 2004 年 3 月至 2020 年 6 月我院机构批准的心脏肿瘤数据库，以寻找心脏副神经节瘤病例。这些前瞻性收集的数据进行了回顾性分析。为每位患者和汇总统计数据呈现了患者特征。还报告了人口统计学和临床数据，包括连续变量的中位数和四分位距以及分类变量的频率和比例。Kaplan-Meier 曲线用于描绘手术患者的生存情况。
共 21 例原发性心脏副神经节瘤，其中 19 例行手术切除，3 例拒绝手术。19 例切除的肿瘤中，13 例起源于左心房，6 例起源于肺动脉和主动脉根部。需要进行复杂的手术，包括主动脉和肺动脉根部置换以及 8 例自体移植。所有肿瘤均行大体全切除，无明显残留肿瘤，但其中 4 例镜下切缘阳性。无患者发生局部肿瘤复发。有 1 例转移性副神经节瘤患者，术后 4 年死亡。手术死亡率为 10.6%。手术生存率分别为 88.2%、71.8%和 71.8%，1、5 和 10 年生存率分别为 1、5 和 10 年。
心脏副神经节瘤具有手术挑战性。手术切除后的死亡率和长期生存率是可以接受的，但可能需要复杂的切除和重建。
