Pericardial synovial sarcoma presenting with large recurrent pericardial effusion

Abstract

Primary pericardial synovial sarcoma is an extremely rare disease with a dismal prognosis. Its main presenting symptoms are a large pericardial effusion, signs of cardiac tamponade, and visualization of a pericardial mass on echocardiography. However, the systemic symptoms of fever, cough, and night sweats may present a clinical picture without any apparent pericardial mass on diagnostic imaging, potentially impeding the diagnosis. We report the case of a 34-year-old patient with fever and recurrent pericardial effusion for 2 years, who was diagnosed with primary pericardial synovial sarcoma after 2-year follow-up echocardiography.

Keywords: Synovial sarcoma; heart; pericardial effusion; pericardium.

Figure 1

Parasternal long-axis echocardiographic view showing a large amount of pericardial effusion.

Figure 2

Echocardiography showing a mild amount of pericardial effusion (1.8 cm on the right side).

Figure 3

Chest CT findings. Axial (A) and coronal (B) contrast-enhanced CT images, showing localized pericardial effusion with septation and the thickened pericardium.

Figure 4

Cardiac chest CT angiographic findings. Axial (A) and coronal (B) contrast-enhanced CT images show a large ovoid mass on the right inferior side of the pericardial space, compressing the right atrium.

Figure 5

Surgical excision was performed. (A) The tumor is located in the pericardium; (B) gross specimen shows a large, well-demarcated tumor; (C) photomicrograph shows biphasic synovial sarcoma in which epithelial and spindle-cell components are present (Haematoxylin & eosin, ×100).