Primary Pericardial Synovial Sarcoma: A Case Series and Review of Literature

TL;DR

This paper presents two cases of rare primary pericardial synovial sarcoma in young men and reviews all reported cases to date.

Contribution

The study compiles and summarizes all known cases of primary pericardial synovial sarcoma, including clinical and pathological details.

Findings

Two young men with PPSS presented with different symptoms but both had pericardial masses confirmed by imaging.

FISH testing confirmed the SS18-SSX fusion gene in both cases, a hallmark of synovial sarcoma.

A literature review identified 46 articles specifically on PPSS, highlighting its rarity and aggressive nature.

Abstract

Primary pericardial synovial sarcoma (PPSS) is a rare and aggressive cancer that arises from pluripotent mesenchymal cells of the pericardium. The pathognomonic genetic hallmark is the chromosomal translocation t(X;18)(p11;q11), resulting in the SS18-SSX fusion oncogene, which, down the line, disrupts transcription regulation and chromatin remodeling, ultimately leading to carcinogenesis. In our article, we describe two cases of PPSS in previously healthy young men, managed with multidisciplinary teams, along with a review of the literature of cases reported to date. Both of our patients are young adults, with very different presentations in terms of symptoms, one presenting with shortness of breath (SOB) and the second with chest pain. Both patients had imaging studies that reported a pericardial mass. Ultimately, a diagnostic evaluation was done, with fluorescence in situ hybridization (FISH) confirming primary pericardial synovial sarcoma. Unfortunately, both patients passed away within a week of diagnosis.

A comprehensive search of public databases, including PubMed and Google Scholar, was conducted up to 2025. The search criteria included synovial pericardial sarcoma, sarcoma of the heart, cardiac sarcoma, and primary pericardial synovial sarcoma. The search yielded over 100 results, of which 46 articles focused specifically on primary pericardial synovial sarcoma. This article summarizes all reported PPSS cases to date, including patient age, race, gender, symptoms, cytological analysis, and histological subtypes.

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