Primary Intravascular Synovial Sarcoma: Case Report
DOI:
https://doi.org/10.1532/HSF98.20111159Abstract
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.References
Coen M, Gagliano T, Tsolaki E, Marchetti G, Marchetti E, Mascoli F. 2008. Primary intravascular synovial sarcoma of the femoral vein in a male patient, case report. Eur J Vasc Endovasc Surg 36:224-6.nDeshmukh R, Mankin HJ, Singer S. 2004. Synovial sarcoma: the importance of size and location for survival. Clin Orthop Relat Res Feb(419):155-61.nFisher C, Folpe AL, Hashimoto H, Weiss SW. 2004. Intra-abdominal synovial sarcoma: a clinicopathological study. Histopathology 45:245-53.nFisher C, de Bruijn DRH, Geurts van Kessel A. 2002. Synovial sarcoma. In: Fletcher CDM, Unni KK, Mertens F, eds. World Health Organization classification of tumours: pathology and genetics of tumours of soft tissue and bone. Lyon, France: IARC Press. p 200-4.nMiettinen M, Santavirta S, Slätis P. 1987. Intravascular synovial sarcoma. Hum Pathol 18:1075-7.nRobertson NJ, Halawa MH, Smith ME. 1998. Intravascular synovial sarcoma. J Clin Pathol 51:172-3.nShaw GR, Lais CJ. 1993. Fatal intravascular synovial sarcoma in a 31-year-old woman. Hum Pathol 24:809-10.nSuster S, Moran A. 2005. Primary synovial sarcomas of the mediastinum: a clinicopathologic, immunohistochemical, and ultrastructural study of 15 cases. Am J Surg Pathol 29:569-78.nTong GX, Goldblum JR, Qiu W, et al. 2006. Primary intravascular synovial sarcoma: a disease of young adult women? Report of a case diagnosed by aspiration biopsy and review of the literature. Diagn Cytopathol 34:834-8.nWeiss SW, Goldblum JR. 2001. Enzinger and Weiss's soft tissue tumors. 4th ed. St. Louis: Mosby.nWhite JS, Medicott SA, Brown H, Moore R, Temple W. 2005. Intravascular synovial sarcoma of the external iliac vein and reconstruction with the superficial femoral vein. J Vasc Surg 42:365-7.n