原发性心脏血管肉瘤患者的临床特征、管理和预后:系统综述。

IF 1.2 Q3 CARDIAC & CARDIOVASCULAR SYSTEMS
Diego Chambergo-Michilot, Gabriel De la Cruz-Ku, Rosalie M Sterner, Ana Brañez-Condorena, Pedro Guerra-Canchari, John Stulak
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引用次数: 0

摘要

原发性心脏血管肉瘤(PCA)是一种侵袭性极强的恶性心脏肿瘤。以往的报告显示,无论采取何种治疗方法,预后都很差,目前尚无共识或指南。由于 PCA 患者的生存期较短,因此有必要澄清这些信息。因此,我们旨在系统回顾临床表现、管理和预后。我们在 PubMed、Scopus、Web of Science 和 EMBASE 中进行了系统检索。我们打算纳入报道 PCA 患者临床特征、管理和预后的横断面研究、病例对照研究、队列研究和系列病例。在研究方法上,我们对病例系列研究采用了乔安娜-布里格斯研究所的批判性评估核对表,对队列研究采用了纽卡斯尔-渥太华量表。我们纳入了六项研究(五项病例系列,一项队列)。平均/中位年龄从 39 岁到 48.9 岁不等。男性居多。最常见的表现为呼吸困难(50%-80%)、心包积液(29% 和 56%)和胸痛(10%-39%)。肿瘤的平均大小从 5.8 厘米到 7.2 厘米不等,大部分位于右心房(70%-100%)。最常见的转移部位是肺(20%-55.6%)、肝(10%-22.2%)和骨(10%-20%)。最常用的治疗方法是切除(22.9%-94%)和新辅助或辅助化疗(30%-100%)。死亡率从64.7%到100%不等。PCA 通常发病较晚,预后较差。我们强烈建议开展多机构前瞻性队列研究,以更好地研究病程和治疗方法,从而为这类肉瘤制定共识、算法和指南。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

Clinical characteristics, management, and outcomes of patients with primary cardiac angiosarcoma: A systematic review.

Clinical characteristics, management, and outcomes of patients with primary cardiac angiosarcoma: A systematic review.

Primary cardiac angiosarcomas (PCA) are highly aggressive malignant heart tumors. Previous reports have shown a poor prognosis regardless of management, and no consensus or guidelines exist. It is necessary to clarify this information since patients with PCA have a short survival. Therefore, we aimed to systematically review clinical manifestations, management, and outcomes. We systematically searched in PubMed, Scopus, Web of Science, and EMBASE. We intended to include cross-sectional studies, case-control studies, cohort studies, and case series that reported clinical characteristics, management, and outcomes of patients with PCA. As a methodological approach, we used the Joanna Briggs Institute Critical Appraisal Checklist for Case Series and the Newcastle-Ottawa Scale for cohorts. We included six studies (five case series, one cohort). The mean/median age ranged from 39 to 48.9 years. Male sex was predominant. The most frequent manifestations were dyspnea (range: 50%-80%), pericardial effusion (29% & 56%), and chest pain (10%-39%). The mean tumor size ranged from 5.8 to 7.2 cm, with the majority of these localized in the right atrium (70-100%). The most common locations of metastasis were the lung (20%-55.6%), liver (10%-22.2%), and bone (10%-20%). Resection (22.9%-94%), and chemotherapy as neoadjuvant or adjuvant (30%-100%) were the most commonly used methods of treatment. Mortality ranged from 64.7% to 100%. PCA often presents late in its course and usually results in poor prognosis. We strongly recommend performing multi-institutional prospective cohorts to better study disease course and treatments to develop consensus, algorithms, and guidelines for this type of sarcoma.

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来源期刊
Journal of Cardiovascular and Thoracic Research
Journal of Cardiovascular and Thoracic Research CARDIAC & CARDIOVASCULAR SYSTEMS-
CiteScore
2.00
自引率
0.00%
发文量
22
审稿时长
7 weeks
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