Primary intravascular synovial sarcoma: case report

Synovial sarcoma (SS), a mesenchymal spindle cell tumor, displays variable epithelial differentiation, including glandular formation, and features a specific chromosomal translocation, t(X;18)(p11;q11). SS accounts for 5% to 10% of soft-tissue sarcomas. These tumors occur mostly in the joints, especially near the knee, but they also occur in other locations. Primary intravascular SS (IVSS) are extremely rare; only 6 well-documented cases have been reported in the English literature. We describe a new case of primary IVSS of the superior vena cava (SVC) in a 16-year-old boy. A transthoracic echocardiogram confirmed a large (4.8 × 4.6 cm) circumscribed mass filling the right atrium, as well as a moderate pericardial effusion. The mass extended from the SVC to the tricuspid valve but did not prevent valve coaptation. Surgery via a transatrial approach revealed a huge mass (8 to 12 cm) attached to the SVC via a 5-mm pedicle. The tumor was excised, and the patient experienced an uneventful postoperative course. Fluorescence in situ hybridization analysis revealed the presence of the SS-specific translocation.