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英文作者:Xiang Jiao1 Chen Bowen2 Feng Long1 Feng Siting1 Yang Bo3 Duan Xiaochun1
单位:1首都医科大学附属北京安贞医院急诊危重症中心,北京100029;2深圳北京大学香港科技大学医学中心生物医学研究所深圳神经结构生物学重点实验室,深圳518036;3首都医科大学附属北京安贞医院主动脉外科中心二区,北京100029
英文单位:1Emergency and Critical Care Center Beijing Anzhen Hospital Capital Medical University Beijing 100029 China; 2Shenzhen Key Laboratory of Neuronal Structural Biology Institute of Biomedical Research Shenzhen Peking University Hong Kong University of Science and Technology Medical Center Shenzhen 518036 China; 3The Second Ward Aortic Surgery Center Beijing Anzhen Hospital Capital Medical University Beijing 100029 China
关键词:原发性心脏恶性肿瘤;肉瘤;存活率
英文关键词:Primarycardiacmalignanttumors;Sarcoma;Survivalrate
目的 探索原发性心脏恶性肿瘤(PCM)在中国人群中的发病情况、治疗方式和生存情况。方法 回顾2003年8月至2025年7月连续22年在首都医科大学附属北京安贞医院就诊的54例PCM患者的病历资料,随访患者治疗及预后。采用R 4.5.2软件进行基线数据分析及生存分析。结果 54例PCM患者病理类型包括肉瘤12例、血管肉瘤7例、恶性间皮瘤5例、横纹肌肉瘤4例、腺癌4例、平滑肌肉瘤3例、滑膜肉瘤2例、炎性肌纤维母细胞瘤2例、淋巴瘤2例、纤维肉瘤1例、软骨肉瘤1例、脂肪肉瘤1例、恶性纤维组织细胞瘤1例、恶性副神经节瘤1例,有8例未明确病理类型。在不同类型恶性肿瘤中,总体男性患病率更高[男性70.4%(38/54)比女性29.6%(16/54),P=0.001],年龄为(46±17)岁,发病年龄集中在31~60岁。49例因出现包括胸闷、胸痛、心悸、呼吸困难、水肿等在内的症状就诊确诊,仅有5例因体检时意外发现确诊。所有肿瘤平均直径为(43.5±26.7)mm。15例接受内科支持治疗,24例接受手术切除,5例接受化疗,8例接受手术切除联合化疗,2例接受心脏移植。随访截止到2026年1月1日,所有恶性肿瘤患者中,2例失访,43例死亡,9例存活。患者中位生存期为12.2个月,1年、3年和5年存活率分别为51.7%、35.1%、17.2%。不同治疗方式下患者存活率差异无统计学意义(Log-rank P=0.12)。结论 PCM是一种罕见的癌症,因高度恶性,其远期预后差生存率低。尽管采用了不同的治疗方式,但患者的预后并无显著改善。
Objective To explore the incidence, treatment modalities and survival status of primary cardiac malignant tumors (PCM) in Chinese population. Methods The medical records of 54 patients with PCM treated at Beijing Anzhen Hospital, Capital Medical University from August 2003 to July 2025 for 22 consecutive years were retrospectively reviewed, and the treatment and prognosis of patients were followed up. R 4.5.2 software was used for baseline data analysis and survival analysis. Results Among the 54 patients with PCM, the pathological types included sarcoma (12 cases), angiosarcoma (7 cases), malignant mesothelioma (5 cases), rhabdomyosarcoma (4 cases), adenocarcinoma (4 cases), leiomyosarcoma (3 cases), synovial sarcoma (2 cases), inflammatory myofibroblastic tumor (2 cases), lymphoma (2 cases), fibrosarcoma (1 case), chondrosarcoma (1 case), liposarcoma (1 case), malignant fibrous histiocytoma (1 case) and malignant paraganglioma (1 case); 8 cases had undefined pathological types.Among different types of malignant tumors, the prevalence was higher in males [70.4%(38/54) in males vs 29.6%(16/54) in females, P=0.001]. The age was (46±17) years, and the onset age was concentrated at 31-60 years. Forty-nine patients were diagnosed after presenting symptoms including chest tightness, chest pain, palpitations, dyspnea and edema, while only 5 cases were incidentally detected during physical examination. The mean diameter of all tumors was (43.5±26.7) mm. Fifteen patients received medical supportive therapy, 24 underwent surgical resection, 5 received chemotherapy, 8 received surgical resection combined with chemotherapy, and 2 underwent heart transplantation. Follow-up ended on January 1, 2026. Among all patients with malignant tumors, 2 were lost to follow-up, 43 died and 9 survived. The median survival time was 12.2 months, and the 1-, 3- and 5-year survival rates were 51.7%, 35.1% and 17.2%, respectively. There was no statistically significant difference in survival rate among patients receiving different treatment modalities (Log-rank P=0.12). Conclusions PCM is a rare cancer with poor long-term prognosis and low survival rate due to its high malignancy. Despite the adoption of various treatment modalities, the prognosis of patients has not been significantly improved.
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