Primary angiosarcoma of the aorta, great vessels, and the heart (original) (raw)

2013, Journal of Vascular Surgery

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. 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 (five males and eight females; mean age, 54 6 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). 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.