主要心脏血管肉瘤（PCA）是心脏肉瘤中最常见的组织学类型，但其罕见性使得规范化治疗方案面临挑战。此外，已发表的研究由于患者数量较小且缺乏随机化，难以建立基于证据的治疗策略。本系统综述旨在整理异质性的已发表数据，并确定与PCA患者治疗结果相关的因素。我们系统地搜索了PubMed和Scopus文献数据库，寻找报道PCA患者临床、治疗和结果数据的原始文章。使用Kaplan-Meier分析计算进展时间和生存时间。使用Log-Rank检验比较无进展生存和总体生存数据。使用Cox比例风险回归模型对生存数据进行单变量和多变量分析。共分析了127项包含162名患者数据的研究。患者队列的中位年龄为45岁，男性比女性更常受到影响。肿瘤主要位于心脏的右侧，中位大小为6厘米。计算得出中位无进展生存时间为5个月，总体生存时间为12个月。根据单变量和多变量分析，年龄、性别和切除边缘对PCA生存没有显著影响。在单变量分析中，初诊时的转移与总体生存率下降相关，尽管在多变量分析中这种影响并不显著。结合手术和辅助化疗放疗的多模式治疗与统计学上显著的生存益处相关。手术单独进行的中位总体生存时间从6个月增加到辅助化疗后的13个月和放疗后的27个月。发现包括手术和辅助化疗放疗在内的多模式治疗对PCA患者提供了最大的生存益处。 © 2023 Elsevier Ltd. 保留所有权利。

Primary cardiac angiosarcoma (PCA) is the most prevalent histological type of cardiac sarcoma but its rarity poses a challenge for standardizing treatment protocols. Moreover, published studies are limited by small patient numbers and lack of randomization, making it challenging to establish evidence-based treatment strategies. This systematic review aims to consolidate the heterogeneous published data and identify factors related to the treatment outcome of PCA patients.The PubMed and Scopus bibliographic databases were systematically searched for original articles reporting clinical, treatment and outcome data for PCA patients. Kaplan-Meier analysis was used to calculate the time to progression and survival. The Log-Rank test was used to compare progression-free and overall survival data. The Cox proportional hazards regression model was used for univariate and multivariate analysis of survival data.A total of 127 studies containing data for 162 patients were analyzed. The median age of the patient cohort was 45 years, with males being 1.5 times more frequently affected than females. Tumors were primarily located on the right side of the heart, with a median size of 6 cm. Median progression-free and overall survival of 5 months and 12 months, respectively, were calculated. Age, sex, and resection margins did not have a significant impact on PCA survival, as determined by both univariate and multivariate analyses. The presence of metastases at diagnosis was associated with lower overall survival in univariate analysis, although this effect was not significant in multivariate analysis. Multimodality treatment that incorporated surgery and adjuvant chemo-radiotherapy was associated with a statistically significant survival benefit. Median overall survival increased from 6 months with surgery alone to 13 months and 27 months with adjuvant chemotherapy and chemo-radiotherapy, respectively.Multimodality treatment including surgery and chemo-radiotherapy was found to offer the greatest survival benefit for PCA patients.Copyright © 2023 Elsevier Ltd. All rights reserved.