Background: Primary cardiac tumors (PCTs) are rare entities, with only a minority being malignant and capable of distant dissemination. Among the rarest and most challenging metastatic events are brain metastases originating from cardiac tumors. Due to the heart’s direct access to systemic circulation, even benign tumors such as atrial myxomas may cause cerebral embolic phenomena. Understanding the distinct biological behavior, diagnostic pathways, therapeutic strategies, and prognostic implications of these cases remains limited by the scarcity of the available literature.Methods: A systematic review was conducted following PRISMA guidelines. PubMed, Scopus, Embase, and other major databases were systematically searched using specific MeSH terms and keywords related to cardiac tumors and brain metastases. After applying strict inclusion and exclusion criteria, nineteen studies were included, comprising sixteen single-patient case reports and three multi-patient series. Extracted data included tumor histology, cardiac and brain imaging findings, neurological presentation, treatment approaches, and patient outcomes.Results: A total of 320 patients were analyzed. Atrial myxomas represented the predominant benign tumors causing embolic cerebral events, while angiosarcomas and other cardiac sarcomas were responsible for true hematogenous brain metastases. Brain involvement was frequently hemorrhagic and manifested with seizures, focal deficits, or signs of intracranial hypertension. Cardiac echocardiography and cardiac magnetic resonance imaging (CMR) were essential for tumor detection, while brain MRI, including SWI and DWI sequences, and CT scanning were critical for cerebral lesion characterization. Treatment strategies varied according to tumor type and included surgery, radiotherapy, and systemic therapies. Malignant cardiac tumors correlated with a poor prognosis, with median survival post-CNS involvement ranging from 12 to 14 months.Conclusions: Brain metastases from PCTs, though rare, represent a distinct and serious clinical phenomenon. Benign tumors like myxomas mainly cause embolic cerebral events, whereas malignant tumors, particularly sarcomas, lead to true metastatic brain lesions. Recognizing this biological distinction is crucial for diagnosis, prognostication, and therapeutic planning. An integrated multidisciplinary approach combining advanced cardiac and neuroimaging techniques is vital for early detection and appropriate management. Despite multimodal treatment, survival remains limited, underscoring the urgent need for novel targeted therapies and improved surveillance strategies.
背景:原发性心脏肿瘤(PCTs)较为罕见,其中仅少数为恶性且可能发生远处播散。心脏肿瘤引发的脑转移属于最为罕见且最具挑战性的转移事件之一。由于心脏直接连接体循环,即使是心房黏液瘤等良性肿瘤也可能引发脑栓塞事件。目前相关文献稀缺,限制了人们对这类病例独特的生物学行为、诊断路径、治疗策略及预后影响的深入理解。 方法:本研究遵循PRISMA指南进行系统综述。通过PubMed、Scopus、Embase等主要数据库,系统检索与心脏肿瘤及脑转移相关的特定MeSH术语及关键词。经严格纳入与排除标准筛选后,共纳入19项研究,包括16篇单病例报告和3篇多病例系列研究。提取数据涵盖肿瘤组织学类型、心脏与脑部影像学表现、神经系统临床表现、治疗方案及患者预后。 结果:共分析320例患者。心房黏液瘤是导致栓塞性脑事件的主要良性肿瘤,而血管肉瘤及其他心脏肉瘤则引发真正的血行性脑转移。脑部受累常表现为出血性病变,临床可见癫痫发作、局灶性神经功能缺损或颅内高压体征。心脏超声心动图和心脏磁共振成像(CMR)对肿瘤检测至关重要,而脑部MRI(包括SWI和DWI序列)及CT扫描对脑部病变定性具有关键作用。治疗方案因肿瘤类型而异,包括手术、放疗及全身性治疗。恶性心脏肿瘤预后较差,中枢神经系统受累后中位生存期为12至14个月。 结论:原发性心脏肿瘤引发的脑转移虽属罕见,却是一种独特且严重的临床现象。黏液瘤等良性肿瘤主要引发栓塞性脑事件,而恶性肿瘤(特别是肉瘤)则导致真正的转移性脑病变。认识这种生物学差异对诊断、预后判断及治疗规划至关重要。结合先进心脏与神经影像技术的多学科综合诊疗模式对早期发现和规范管理具有重要意义。尽管采用多模式治疗,患者生存期仍然有限,这凸显了开发新型靶向疗法和完善监测策略的迫切需求。