Abstract
Angiosarcoma is a rare, aggressive malignancy originating from endothelial cells and associated with a poor prognosis. Diagnosis is histologically challenging. We present a rare case of cardiac angiosarcoma involving multiple presentations and investigations, which were inconclusive until a diagnosis was finally reached on a biopsy of a cutaneous lesion. Clinical and radiologic response to systemic anthracycline-based chemotherapy is discussed. This is the first reported case of right atrial primary cardiac angiosarcoma presenting with cardiac symptoms rather than systemic symptoms. The case illustrates the challenges associated with the diagnosis of this rare malignancy. It also highlights the feasibility of using anthracycline-based chemotherapy despite the potential cardiotoxicity of these agents.