Key result
Primary pericardial synovial sarcoma recurs at ~11 months with poor prognosis despite surgery and chemoradiotherapy.
Primary pericardial synovial sarcoma is a rare, aggressive cancer with a very poor prognosis, as demonstrated by rapid mortality in two young male patients.
Raises awareness for PPSS in young men with pericardial masses; extends sparse case literature but leaves optimal management open.
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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Kaur et al. (2025) conducted a review in Primary Pericardial Synovial Sarcoma (n=48). Multimodality therapy (surgery, chemotherapy, radiotherapy) was evaluated. Primary pericardial synovial sarcoma is an aggressive and rare malignancy with a high recurrence rate at an average of 11 months and poor overall prognosis despite surgical resection and chemoradiotherapy.
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