Pericardial synovial sarcoma: a case report and review of the literature

Abstract

Primary pericardial synovial sarcoma is a rare disease. We herein report a case of synovial sarcoma that originated in the epicardium. A 13-year-old male visited our hospital with a fever and chest pain. Copious pericardial effusion and a large intrapericardial tumor were detected. An open-chest tumor resection was performed. A solid nodular tumor was observed in the pericardial cavity. The tumor was a polypoid mass that was pedunculated and grew from the inner surface of the pericardium near the origin of the SVC and ascending aorta. Histologically, the tumor cells were uniformly spindle shaped, with an ovoid or oval nucleus, and formed solid, compact sheets and fascicles. A storiform pattern was also observed. Based on the histopathological and immunohistochemical findings, and the fluorescence in situ hybridization detection of rearrangement of the SYT gene, a monophasic synovial sarcoma was diagnosed. We discuss the diagnosis and treatment of this case and review the pertinent literature.

Pericardial synovial sarcoma: challenges in diagnosis and management

“…They are classified as biphasic, monophasic and poorly differentiated based on their histology. Among the pericardial synovial sarcomas, the biphasic type (composed of an epithelial component and a spindle-cell component) appears to be more common than the monophasic type (only spindle-cells) and the poorly differentiated type (small round cells) 2, 9– 12 . Our patient had a monophasic variant with predominant spindle to oval cells in a fascicular pattern.…”

“…The real incidence of pericardial synovial sarcoma may be underestimated though, as the precise site of origin of such tumors can be difficult to determine. To date, to the authors’ knowledge, only 20 cases have been reported in the English literature 2 .…”

“…It is an aggressive tumor with a poor prognosis. Most patients have a short survival time 1, 2 . One exception is the case report of a patient whose survival exceeded 14 years and involved recurrences and multiple surgical resections 4 .…”

“…Although termed as synovial sarcoma, these tumors have now been recognized to arise from mesenchymal tissue and show epithelial differentiation 1 . To the authors’ knowledge, only 20 cases of synovial sarcomas originating from the pericardium have been described in the English literature to date 2 . This type of tumor is known to have a poor prognosis with no clarity about the best approach in managing this disease.…”

Pericardial sarcoma

“…Pericardiocentesis and cytological analysis of pericardial fluid is usually non-diagnostic and lacks specificity 9. Imaging modalities are usually the cornerstone for diagnosis 8 10.…”