Young Heart, Rare Tumor: A Case Report On Pericardial Synovial Sarcoma

Abstract

Pericardial synovial sarcoma [PSS] is a sporadic rare primary malignant tumour of the heart. It belongs to a category of synovial sarcoma [SS] which accounts for approximately 5-10% of all soft tissue sarcomas and 4^th most common sarcoma. Mostly occur in people aged 20 to 40 years [1]. SS usually occurs in the extremities near joints, though it can rarely appear in other sites such as thoracic cavity [2]. Patients usually present with a deep-seated mass that has been present for several years. Most synovial sarcoma show a characteristic chromosomal translocation t(x;18) (p11;q11.2) producing SS18-SSX1-SSX2 or SSX4 fusion genes that encode chimeric transcription factors [3]. Histologically PSS may be monophasic, characterized by uniform spindle cells, or biphasic, containing both spindle and epithelial components. The diagnosis of PSS can be challenging due to its non-specific presentation. Today we will discuss one such case.