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

• Published in: Interactive cardiovascular and thoracic surgery Vol. 15; no. 5; pp. 909 - 911
• Main Authors: Loggos, Spiros, Kondrafouris, Konstandinos, Oikonomopoulos, George, Mitropoulos, Fotios
• Format: Journal Article
• Language: English
• Published: England Oxford University Press 01.11.2012
• Subjects: Adolescent; Cardiac Surgical Procedures; Cardiopulmonary Bypass; Case Reports; Circulatory Arrest, Deep Hypothermia Induced; Disease Progression; Fatal Outcome; Heart Atria - pathology; Heart Atria - surgery; Heart Ventricles - pathology; Heart Ventricles - surgery; Humans; Male; Mediastinal Neoplasms - complications; Mediastinal Neoplasms - pathology; Mediastinal Neoplasms - surgery; Multiple Organ Failure - etiology; Neoplasm Invasiveness; Reconstructive Surgical Procedures; Sarcoma, Synovial - complications; Sarcoma, Synovial - pathology; Sarcoma, Synovial - surgery; Time Factors; Treatment Outcome; Tumor Burden; Vena Cava, Inferior - pathology; Vena Cava, Inferior - surgery; Radical surgical excision; Monophasic synovial sarcoma
• ISSN: 1569-9293; 1569-9285
• DOI: 10.1093/icvts/ivs324

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.