Ann Thorac Surg 2009;87:322. doi:10.1016/j.athoracsur.2008.03.067
© 2009 The Society of Thoracic Surgeons
Images in Cardiothoracic Surgery
Primary Cardiac Synovial Sarcoma With Imminent Tricuspid Valve Obstruction
Ralph W. White, FRCS (CTh)a,*,
Jeremy Rushbrook, MRCSa,
Mohan U. Sivananthan, MDb,
Joseph P. McGoldrick, MDa
a Department of Cardiothoracic Surgery, Yorkshire Heart Centre, Leeds General Infirmary, Leeds, United Kingdom
b Department of Cardiology, Yorkshire Heart Centre, Leeds General Infirmary, Leeds, United Kingdom
* Address correspondence to Dr White, Department of Cardiothoracic Surgery, General Infirmary, Great George St, Leeds, LS1 3EX, United Kingdom (Email: ra1ph69{at}hotmail.com).
A 22-year-old man presented with dyspnea and was found to have a large pericardial effusion. Pericardiocentesis was performed, and 500 mL of bloodstained fluid was drained. A chest roentgenogram showed cardiomegaly, and a subsequent computed tomographic scan revealed a right atrial mass, with evidence of caval involvement, but without evidence of metastasis. A magnetic resonance imaging scan (Fig 1;
LA = left atrium, LV = left ventricle, RV = right ventricle) accurately demonstrated a large right atrial epicardial mass (white arrowheads) with intra-atrial extension (arrows) and demonstrates protrusion into the SVC (Fig 2;
RA = right atrium, RV = right ventricle, SVC = superior vena cava; black arrowheads). Thorough preoperative collaboration between the cardiac surgery, cardiology, and radiology departments was used to plan and implement the best treatment for suspected cardiac malignancy, even though preoperative diagnosis was not established. At operation, a lobulated tumor covered the surface of the right atrium, and the intracardiac portion almost completely filled the right atrium; tricuspid valve obstruction was imminent. With the excellent preoperative imaging, safe and successful surgical excision was facilitated, using cardiopulmonary bypass, but without cardioplegia or circulatory arrest. Figure 3
shows the surgical specimen, with the intracardiac portion on the left side of the photograph and the larger extracardiac portion on the right side. Almost the whole free wall of the right atrium was resected, which was then reconstructed with bovine pericardium. Formal histology revealed a primary cardiac synovial sarcoma. Unfortunately, the resection margin (at the inferior vena cava) was not free of tumor. The patient was discharged home without complication on postoperative day 5 for planned adjuvant chemoradiotherapy.
Primary cardiac synovial sarcoma is a rare malignancy, comprising approximately 5% of cardiac sarcomas and less than 0.1% of all primary cardiac tumors, and it has a poor prognosis. However, surgical resection (excision) offers the best hope of accurate diagnosis and effective palliation.
