Primary right atrial angiosarcoma mimicking acute pericarditis, pulmonary embolism, and tricuspid stenosis

Abstract

A 29 year old white man presented to the emergency room with new onset pleuritic chest pain and shortness of breath. He was initially diagnosed as having viral pericarditis and was treated with non-steroidal anti-inflammatory drugs. A few weeks later he developed recurrent chest pain with cough and haemoptysis. Chest radiography, cardiac examination, transthoracic and transoesophageal echocardiography pointed to a mass that arose from the posterior wall of the right atrium, not attached to the interatrial septum, which protruded into the lumen of the right atrium causing intermittent obstruction of inflow across the tricuspid valve. Contrast computed tomography of the chest showed a right atrial mass extending to the anterior chest wall. The lung fields were studded with numerous pulmonary nodules suggestive of metastases. A fine needle aspiration of the pulmonary nodule revealed histopathology consistent with spindle cell sarcoma thought to originate in the right atrium. Immunohistochemical stains confirmed that this was an angiosarcoma. There was no evidence of extracardiac origin of the tumour. The patient was treated with chemotherapy and radiation. This case highlights the clinical presentation, rapid and aggressive course of cardiac angiosarcomas, and the diagnostic modalities available for accurate diagnosis.