Primary Cardiac Osteosarcoma in Right Ventricular Outflow Tract
A 30-year-old woman was referred to our clinic because of shortness of breath and general fatigue. She had been well until 2 months earlier, when she noticed mild shortness of breath during and after exercise. Physical examination revealed a systolic heart murmur at the left parasternal area. Transthoracic echocardiography demonstrated an abnormal mass located just proximal to the pulmonary valve occupying the right ventricular outflow tract (Figure 1⇓, top left). There was a small pericardial effusion, and a flattened ventricular septum suggested elevated right ventricular pressure. The regionally magnified image indicated relatively low echogenicity within the mass (Figure 1⇓, top right). This mass was attached to the side of the right ventricular free wall and was free from the pulmonary valve. Transesophageal echocardiography demonstrated the mass in the right ventricular outflow tract with accelerated color flow across the mass, suggesting hemodynamically significant obstruction (Figure 1⇓, bottom). Ultrasound and radiographic examinations did not demonstrate any other primary lesions in the abdominal or genital organs. One week later, radical resection of the mass and reconstruction of the right ventricular outflow tract were performed. The tumor originated from the right ventricular outflow tract. A small part of it had extended beyond the pulmonary valve into the main pulmonary artery. The mass seemed to be a myxoma (Figure 2⇓, left), but microscopic examination of hematoxylin-eosin–stained specimens showed a uniform population of large atypical cells with prominent nucleoli. Interestingly, there was evidence of an osteogenic sarcomatous element (Figure 2⇓, right). Immunohistochemical stains were negative for such antigens as myoglobin, α-actin, S-100, factor VIII, CD31, and CD34. Thus, the tumor was considered to be an osteosarcoma, which constitutes only 0% to 3.4% of cardiac tumors.1 2
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