Clinical and radiologic characteristics as well as outcomes of patients with primary cardiac angiosarcoma

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.