Department of Radiation Oncology, Stanford University School of Medicine, Stanford, Calif.
Department of Pathology, Stanford University School of Medicine, Stanford, Calif.
J Thorac Cardiovasc Surg. 2018 Dec;156(6):2194-2203. doi: 10.1016/j.jtcvs.2018.04.127. Epub 2018 Jun 6.
Primary cardiac sarcomas (PCS) are extremely rare, portend a very poor prognosis, and have limited outcomes data to direct management. This study evaluated the impact of postoperative chemotherapy and/or radiotherapy on survival for PCS.
A retrospective chart review was conducted of 12 patients diagnosed with and who underwent resection for PCS at a single institution between 2000 and 2016. Data were collected on patient/tumor characteristics and analyzed with respect to treatment and outcome using Kaplan-Meier methods.
Median age was 43 (range 21-73 years) with a 50:50 male-to-female ratio. The most common subtype was angiosarcoma (42%), and 25% presented with distant metastases (DMs). The initial treatment modality for all patients was surgery, with 58% having macroscopically positive (R2) margins. In total, 75% received postoperative chemotherapy and/or radiotherapy. Median progression-free survival (PFS) was 5.9 months, and median overall survival (OS) was 12.0 months. Achieving negative or microscopically positive margins (R0/R1) as compared with R2 resection significantly improved PFS (12.6 vs 2.7 months, P = .008) and OS (21.8 vs 7.2 months, P = .006). DM at presentation demonstrated a significantly shorter OS (7.0 vs 16.9 months, P = .04) and PFS (0.7 vs 7.9 months, P = .003) compared with localized disease. Patients given postoperative therapy had longer OS compared with surgery only, but this difference was not statistically significant (15.5 vs 2.6 months, P = .12).
Gross total surgical resection can significantly improve PFS and OS in PCS, but DM at diagnosis is an extremely poor prognostic sign. Postoperative therapy should be considered, although this study was likely underpowered to demonstrate a statistically significant benefit.
原发性心脏肉瘤（PCS）极为罕见，预后极差，目前针对其管理的研究数据有限。本研究旨在评估术后化疗和/或放疗对 PCS 患者生存的影响。
对一家机构于 2000 年至 2016 年间确诊并接受手术切除治疗的 12 例 PCS 患者进行回顾性图表审查。收集患者/肿瘤特征数据，并采用 Kaplan-Meier 方法分析治疗和结果。
中位年龄为 43 岁（范围 21-73 岁），男女比例为 50:50。最常见的亚型是血管肉瘤（42%），25%的患者存在远处转移（DM）。所有患者的初始治疗方法均为手术，58%的患者存在肉眼阳性（R2）切缘。共有 75%的患者接受了术后化疗和/或放疗。中位无进展生存期（PFS）为 5.9 个月，中位总生存期（OS）为 12.0 个月。与 R2 切除相比，获得阴性或镜下阳性（R0/R1）切缘可显著改善 PFS（12.6 与 2.7 个月，P=0.008）和 OS（21.8 与 7.2 个月，P=0.006）。与局限性疾病相比，初诊时存在 DM 的患者 OS（7.0 与 16.9 个月，P=0.04）和 PFS（0.7 与 7.9 个月，P=0.003）明显缩短。接受术后治疗的患者 OS 长于单纯手术治疗，但差异无统计学意义（15.5 与 2.6 个月，P=0.12）。
完全手术切除可显著改善 PCS 的 PFS 和 OS，但初诊时存在 DM 是一个预后极差的表现。尽管本研究可能没有足够的统计学效力来证明术后治疗具有显著的获益，但仍应考虑术后治疗。
