Differentiating aortic fibrosarcoma from acute intramural hematoma

Abstract

FIGURE 1. CTwith contrast shows the tumor (asterisk) in both long-axis and transverse views. The pericardial effusion is also evident in the transverse view (arrow). An 86-year-old woman with a medical history of hypertension was transferred to the Hennepin County Medical Center for severe substernal chest pain radiating to her back, associated with coughing. Physical examination revealed normal heart sounds with irregular heart rate, symmetric pulses, and intact neurologic examination. Electrocardiogram on admission showed atrial fibrillation. Chest computed tomography (CT) with contrast obtained before her transfer demonstrated soft tissue surrounding her ascending aorta, which was diagnosed as an aortic intramural hematoma (Figure 1) and a small pericardial effusion. An aortic dissection flap was not observed. Transthoracic echocardiography before emergency surgical exploration showed a soft tissue collection posterior to the ascending aorta without a dissection flap and a small pericardial effusion. On surgical exploration, a large white mass completely surrounding the distal ascending aorta and compressing the superior vena cava was observed. Biopsies from this mass were obtained showing a monomorphic spindle cell sarcoma (Figure 2). Immunostains for a variety of markers were negative, including wide spectrum cytokeratin, cytokeratin 5/6, Wilms’ tumor protein 1, Transducinlike enhancer of split 1, CD34, actin, desmin, and S-100. The morphology and immunohistochemistry were consistent with a high-grade adult-type fibrosarcoma. No other surgical intervention was performed. The patient elected to have her care at a different institution, and therefore no follow-up data are available. On the basis of the above stains, the tumor could be aortic or cardiac in origin.