Complete excision of large pericardial synovial sarcoma

Abstract

Primary cardiac neoplasms have an incidence of <0.05%, with 70% of them being benign and myxomas accounting for the majority. Sarcomas are the most common malignant variants, with pericardial synovial sarcomas representing 3% and only 43 reported cases. They are diagnostically characterized by t(X;18) (p11.2;q11.2) (SYT-SSX) chromosomal translocation in 95% of cases. Frequent clinical manifestations are secondary to embolization, haemodynamic obstruction, direct myocardial invasion and pericardial effusions causing tamponade. Complete surgical resection is difficult, and the resulting prognosis is generally poor despite adjuvant therapies. A 48-year-old Polynesian male presented with fatigue, night sweats and atrial flutter. Computed tomography and echocardiogram identified a large pericardial mass compressing the right atrium (Fig. 1). Cardiac magnetic resonance imaging revealed that the mass was invading into the pericardium and inferior vena cava (IVC), with position emission topography excluding metastatic disease. Thoracoscopic biopsy confirmed a biphasic synovial sarcoma. The patient underwent sternotomy, and complete en bloc excision of the tumour with macroscopic clearance was achieved in conjunction with a right middle lobe wedge resection, IVC resection and preservation of the right atrial wall (Figs 2,3a). IVC reconstruction was then conducted under deep hypothermic circulatory arrest. Histopathology indicated that the 125 × 60 × 35 mm tumour infiltrated IVC luminal endothelium, but the lung and atrial wall margins were clear. Staining was positive for AE1/AE3 and EMA, as well as