Angiosarcoma of the Pericardium
A Fatal Disease
A 31-year-old male presented with increasing exertional breathlessness. He had been healthy previously, with no recent chest pain or fever. The only notable abnormalities on blood testing were a microcytic anemia and an elevated lactate dehydrogenase level. The chest radiograph showed an increased cardiothoracic ratio and mild pulmonary congestion. Transthoracic echocardiography showed reasonable left ventricular systolic function and chamber sizes within normal limits. Doppler study disclosed marked respiratory variations in transmitral flow without significant regurgitation. In the pericardium, a homogenous mass was seen surrounding the heart, but mainly posterolaterally, and there was minimal pericardial effusion (Figure 1). His clinical situation deteriorated quickly, and he was sent for emergency thoracotomy. A large mass, which surrounded the entire heart but did not invade the myocardium, was discovered and partially removed (Figure 2). One week after an initial clinical improvement, the patient developed signs of tamponade again. Cardiac MRI showed a mass surrounding both ventricles, atria, the ascending aorta, and the pulmonary trunk (Figure 3). Unfortunately, the patient died a few days later owing to respiratory insufficiency. The final histological diagnosis was high-grade sarcoma that corresponded to an angiosarcoma.
Angiosarcomas are the most common primary cardiac malignant tumor. In autopsy series, the cumulative prevalence of cardiac angiosarcoma is 0.03% to 0.0002%. They tend to be found in patients aged 20 to 50 years. Angiosarcomas respond poorly to chemotherapy. Most patients do not survive beyond 6 months of diagnosis.