Large monophasic synovial sarcoma of the mediastinum in a 15-year old boy

Abstract

We present the interesting case of a 15-year old boy with a monophasic synovial sarcoma (MSS) of the mediastinum, which was infiltrating the right heart chambers and the inferior vena cava (IVC). A radical excision was performed, with extensive reconstruction of the heart, under deep hypothermic circulatory arrest. Radical surgical excision is considered to be the treatment of choice for these lesions, as chemotherapy and radiotherapy have little effect. Unfortunately, the patient and his parents refused any further consultation with an oncologist and, although there was no recurrence at 12 months following the procedure, at 24 months we were informed of his death due to the tumour appearing on the left cardiac chambers with subsequent multi-organ failure.

Figure 1:

There were no signs of the lesion infiltrating the left heart chambers and, finally, the right phrenic nerve was also affected.

Figure 2:

(a) For the complete and radical resection of the tumour. (b) The excised mass was 14 × 8, 5 × 6 cm. (c) We finally used the rest of the Peri-Guard® patch to reconstruct the right atrium.