title: Primary intravascular synovial sarcoma: case report.
abstract: 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.
authors.affiliation: Department of Cardiovascular Surgery, Faculty of Medicine, Akdeniz University, Antalya, Turkey. dr_ osmantuncer@hotmail.com
authors.forename: Osman Nuri
authors.initials: ON
authors.lastname: Tuncer
authors.forename: Ozan
authors.initials: O
authors.lastname: Erbasan
authors.forename: Ilhan
authors.initials: I
authors.lastname: Golbasi
mesh_terms: D000293:Adolescent; D002315:Cardiopulmonary Bypass / Q000379:methods; D004452:Echocardiography; D004636:Emergency Service, Hospital; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D035583:Rare Diseases; D018570:Risk Assessment; D013584:Sarcoma, Synovial / Q000175:diagnosis* / Q000601:surgery*; D056346:Sternotomy; D014057:Tomography, X-Ray Computed / Q000379:methods; D016896:Treatment Outcome; D019043:Vascular Neoplasms / Q000175:diagnosis* / Q000601:surgery*; D014683:Vena Cava, Superior / Q000473:pathology* / Q000601:surgery