Primary monophasic mediastinal, cardiac and pericardial synovial sarcoma: a young man in distress

Abstract

A 19-year-old male was admitted because of exertional dyspnoea. The imaging studies revealed epicardial, pericardial and mediastinal masses. The tumours could not be resected through a minor thoracotomy, only biopsies could be taken. Analyses led to the final diagnosis of a monophasic synovial sarcoma. The patient preferred a conservative and palliative approach. Three months later he died at home. Autopsy demonstrated dramatic extension of the tumour masses. We conclude this report with a discussion on primary cardiac tumours. (Neth Heart J 2007;15:226-8.).

Figure 1

Echocardiogram: parasternal short-axis view showing pericardial effusion (PE), a pericardial mass (arrow head) and multiple epicardially located tumours (arrows).

Figure 2

MRI: transversal plane (balanced FFE sequence) showing pericardial effusion (PE), bilateral pleural effusion (PlE), atelectasis of the left lung (arrow head) and a tumorous mass encircling the aorta (Ao) and pulmonary artery (PA) (arrows).

Figure 3

Histopathological analysis showing uniform spindleshaped tumour cells arranged in bundles, invading the microvasculature.

Figure 4

Autopsy showing a tumour encircling the heart, extending to the left lung and left thoracic wall (arrow ←), penetrating the right-sided diaphragm, reaching the hepatic surface (arrow ↗) and a solitary tumour in the pericardium (arrow ↙).