Primary pericardial synovial sarcoma presenting as cardiac tamponade

Abstract

Primary malignant cardiac neoplasms are a rare entity. Malignant pericardial tumors may present with pericardial 
effusion or non-specific symptoms. We report about a middle-aged male patient who presented with cardiac 
tamponade and was diagnosed with biphasic phenotype of primary pericardial synovial sarcoma, using histopathology 
and immunohistochemistry. Diagnosis of pericardial synovial sarcoma requires a multipronged approach involving 
clinical, imaging, histopathological and cytogenetic evaluation. Demonstration of a specific cytogenetic alteration 
(chromosomal translocation t(X;18)(p11.2;q11.2) SYT-SSX) remains the ideal confirmatory test. However, in resource 
constrained settings, a detailed histopathological examination combined with immunohistochemistry, specifically 
nuclear staining for Transducin like enhancer of slit-1 in tumor cells is useful for accurate diagnosis, due to its high 
sensitivity and specificity. Early diagnosis followed by prompt initiation of therapy may lead to improved outcomes 
despite the dismal prognosis associated with this neoplasm