​Javairiah Fatima MD, Audra A. Duncan MD, Gustavo S. Oderich MD, Peter Gloviczki MD, Manju Kalra MD, Michael McKusick MD, Thomas C. Bower MD
Mayo Clinic, Rochester, MN.

OBJECTIVES: Primary angiosarcomas originating from the heart, aorta or the great vessels are extremely rare, and hence poorly understood. We reviewed our experience to identify a preferred diagnostic and treatment strategy, and evaluate role of adjunctive therapy.

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.

RESULTS: Thirteen patients (5 males and 8 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). Aortic tumors most commonly presented with lower extremity claudication (n=4), renovascular HTN (n=3), abdominal pain (n=5), and weight loss (n=4). Cardiac and pericardial tumors presented with dyspnea (n=5) due to pleural effusion or cardiac tamponade. All 13 patients underwent Computed Tomography (CT) scan demonstrating irregular, lobulated mass/thrombus with peripheral enhancement and 8 patients had diagnostic echocardiogram. Metastatic disease was present in 10 patients; most common site was lungs (n=6). All except one patient had high grade sarcoma on pathology. Most common endothelial antigen was CD31. Nine patients were treated surgically; resection with aortic reconstruction (n=6), thrombectomy (n=1), and pericardiectomy with patch (n=2). Adjunctive treatment included chemotherapy (n=7) and radiation (n=4). Median survival was 8 months (range 1-75). Patients treated with all 3 treatment modalities had longer survival than patients treated with a single modality (p=0.013). Patients treated with chemotherapy had a more favorable survival than those without chemotherapy (p=0.048).

CONCLUSIONS: Primary angiosarcoma of the heart and great vessels is rare, but a harbinger of poor prognosis. Endothelial antigen CD31 is diagnostic. Combined therapy with surgical resection and chemoradiotherapy offer patients the best survival.

AUTHOR DISCLOSURES: J. Fatima, Nothing to disclose; A. Duncan, Nothing to disclose; G. Oderich, Clinical trial, National Principal Investigator, T-branch stent graft trial, Cook Medical Inc.; P. Gloviczki, Nothing to disclose; M. Kalra, Nothing to disclose; M. McKusick, Nothing to disclose; T. Bower, Nothing to disclose.

Posted April 2012