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Title: [Clinical and radiologic characteristics as well as outcomes of patients with primary cardiac angiosarcoma].
Author: Zhang ZY, Gao X, Bai CM, Zhang SY, Miao Q.
Journal: Zhonghua Xin Xue Guan Bing Za Zhi; 2019 Sep 24; 47(9):731-736. PubMed ID: 31550845.
Abstract:
Objective: To analyze the clinical, radiologic characteristics, and outcome of consecutive patients with primary cardiac angiosarcoma. Methods: The medical records of patients with primary cardiac angiosarcoma diagnosed through pathology at Peking Union Medical College Hospital between January 2001 and May 2018 were obtained. The results of echocardiography, coronary artery CT angiography (CTA), cardiac magnetic resonance (CMR), positron emission computed tomography (PET-CT), operation, postoperative treatment and prognosis of the patients were analyzed. Results: A total of 16 patients were included, 9 were male, 7 were female, the median age was 42.5 years (31.7, 52.5). The interval from symptoms onset to diagnosis was 4.5 months (0.5-18.0 months). Eight patients were diagnosed at non-metastatic phase, while 8 patients were in metastatic phase. The main complaints were dyspnea, short breath, chest pain, hemoptysis, syncope, edema, fever, fatigue, and cough. Three patients (18.8%) had pericardial tamponade in the course of the disease. Echocardiography was performed on all 16 patients, cardiac mass was found in 12 patients, and pericardial effusion or pericardial thickening was observed in 4 patients. In 8 cases with results of coronary CTA, 5 cases presented signs of right atrium occupation, 1 case presented sign of right ventricle occupation, and 1 case presented isolated massive pericardial effusion. None abnormality was found in 1 case. Of the 7 patients who underwent CMR, 6 presented with right atrium mass occupation and 1 mediastinal mass. Four patients received PET-CT examination and results showed that all presented with hypermetabolic lesions: 2 in right atrium, 1 in pericardium, and 1 in mediastinum, and lung metastasis was found in 2 cases. Among the 16 patients, 13 received surgical treatment, 2 received adjuvant chemotherapy, and 1 received chemotherapy and radiotherapy after biopsy. The median overall survival was 3.0 months. Conclusion: Cardiac angiosarcoma is a rare malignancy with poor prognosis, and echocardiography has only limited diagnostic value for angiosarcoma. CMR, CTA or PET-CT examinations could provide valuable clues for the diagnosis of this rare disease. 目的： 分析心脏原发性血管肉瘤的临床影像学特征及预后。 方法： 回顾性分析2001年1月至2018年5月在北京协和医院收治病理确诊的心脏原发性血管肉瘤患者共16例，查找病历及影像学资料，分析其超声心动图、冠状动脉CT血管成像（CTA）、心脏磁共振（CMR）、正电子发射计算机断层显像（PET-CT）等影像学结果，以及患者手术、术后治疗及预后情况。 结果： 16例患者中，男9例，女7例，确诊时年龄42.5（31.7，52.5）岁，从症状出现至确诊时间为4.5（0.5~18.0）个月。确诊时8例为局限性肿瘤，8例有远处转移，主要临床表现为胸闷憋气、气短、胸痛、咯血等，3例患者发生过心包填塞。16例患者均行超声心动图检查，12例发现心脏占位，4例表现为心包积液或心包增厚。8例行冠状动脉CTA，5例为右心房占位，1例为右心室占位，1例仅表现为大量心包积液，1例无发现。7例患者行CMR检查，6例表现为右心房占位，1例为纵隔占位。4例患者行PET-CT检查均发现代谢增高灶，2例为右心房，1例为心包，1例为纵隔；2例发现肺转移。16例患者中13例行手术治疗，2例术后行辅助化疗，1例活检确诊后行化疗及放疗。16例患者总中位生存期为3.0个月。 结论： 心脏原发性血管肉瘤是罕见的恶性肿瘤，预后不佳；超声心动图对心脏血管肉瘤的诊断价值有限，可同时行CMR、冠状动脉CTA或PET-CT辅助诊断。.

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