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Primary angiosarcoma of the aorta, great vessels, and the heart.

Authors :: Fatima J
Duncan AA
Maleszewski JJ
Kalra M
Oderich GS
Gloviczki P
Suri RM
Bower TC
Source :: Journal of vascular surgery [J Vasc Surg] 2013 Mar; Vol. 57 (3), pp. 756-64. Date of Electronic Publication: 2013 Jan 09.
Publication Year :: 2013
Abstract: Objective: Primary angiosarcomas originating from the heart, aorta, or great vessels are extremely rare and hence poorly understood. We reviewed our experience to identify a preferred diagnostic and treatment strategy and evaluate the role of adjunctive therapy.<br />Methods: We reviewed the clinical data of all patients diagnosed with primary angiosarcoma of the heart, aorta, and great vessels from 1985 to 2011, including presentation, diagnosis, management, and outcomes.<br />Results: Thirteen patients (five males and eight females; mean age, 54 ± 4 years) had primary angiosarcoma arising from the aorta (n = 7), heart (n = 3), pericardium (n = 2), and pulmonary artery (n = 1). Patients with aortic tumors most commonly presented with lower extremity claudication (n = 2), renovascular hypertension (n = 3), abdominal pain (n = 5), and weight loss (n = 4). Patients with cardiac and pericardial tumors presented with dyspnea (n = 5) due to pleural effusion or cardiac tamponade. All 13 patients underwent computed tomographic scan, which demonstrated irregular, lobulated mass/thrombus with peripheral enhancement, and eight patients underwent diagnostic echocardiography. Metastatic disease was present in 10 patients. The most common site was the lungs (n = 6). All except one patient exhibited high-grade morphology histopathologically. Nine patients were treated surgically: resection with aortic reconstruction (n = 5), thromboendarterectomy (n = 2), pericardiectomy/atrial septal resection with patch reconstruction (n = 2), and just biopsy (n = 1). Adjunctive treatment included chemotherapy (n = 6) and radiation (n = 4). Median survival was 8 months (range, 1-75 months). Patients treated with all three treatment modalities had longer survival than did patients treated with a single modality (P = .013). Patients treated with chemotherapy had a more favorable survival than did those without chemotherapy (P = .048).<br />Conclusions: Primary angiosarcoma of the heart and great vessels is rare but is a harbinger of poor prognosis. Pathologic examination is necessary to confirm the diagnosis. Combined therapy with surgical resection and chemoradiotherapy offers patients the best survival.<br /> (Copyright © 2013 Society for Vascular Surgery. Published by Mosby, Inc. All rights reserved.)