A Patient with Primary Pericardial Synovial Sarcoma who Presented with Cardiac Tamponade: A Case Report and Review of the Literature

Abstract

A 36-year-old man presented with near-syncope. He was found to have massive pericardial effusion with a giant pericardial tumorous lesion. The pericardial effusion exhibited a bloody nature; however, neither malignant cells nor infectious organisms were detected.18 F-fluorodeoxyglucose-positron emission tomography (FDG-PET) showed an increased uptake of FDG in the pericardial tumor only. Although the tumor was not resectable, thoracotomy and tissue sampling were performed. A histological analysis showed CD99 positivity and SYT gene rearrangement, leading to a diagnosis of synovial sarcoma arising from the left lateral pericardial surface. The patient is now receiving chemotherapy.

Pericardial synovial sarcoma presenting with large recurrent pericardial effusion

“…We have described the case of a patient with a pericardial synovial sarcoma that had an unusual clinical presentation. Primary pericardial tumor is an extremely rare disorder (1)(2)(3). Pericardial cysts and pericardial lipomas are the most common benign pericardial tumors, while mesothelioma is the most common primary malignant tumor of the pericardium.…”

“…Primary pericardial sarcoma has a large range of histologic subtypes: angiosarcoma, undifferentiated sarcoma, fibrosarcoma, liposarcoma, rhabdomyosarcoma, and synovial sarcoma (5). Among these, pericardial synovial sarcoma is extremely rare; only 20 cases of synovial sarcoma of the pericardium have been reported in the English language literature (2,3). Histologically, synovial sarcoma has three subtypes: biphasic, monophasic, and poorly differentiated types.…”

“…The chest X-ray shows a varying degree of cardiomegaly and echocardiography reveals a large pericardial effusion with a pericardial mass of variable size, and therapeutic or diagnostic pericardiocentesis yields a large quantity of bloody pericardial effusion, but pericardial fluid analysis usually fails to reveal malignant cells (1)(2)(3). As in our case, generalized symptoms, including fever, cough, chest pain, and fatigue, may occur without any pericardial mass being seen on imaging studies; this poses a diagnostic challenge.…”

“…The cytogenetic diagnosis of synovial sarcoma identifies the presence of the fusion gene SS18-SSX by the reciprocal translocation t(X;18)(p11;q11): specifically, SYT-SSX1 fusion transcript in biphasic synovial sarcoma and SYT-SSX2 fusion transcript in monophasic synovial sarcoma (1,3). No effective treatment strategy has been determined because of the tumor's rarity and highly aggressive behavior, which results in a median survival of 25 months (1,2). Complete excision or near total removal was feasible in only 17-22.7% of the reported cases, and adjuvant chemotherapy and radiation therapy are recommended for residual or recurrent tumors (1,2,8,9).…”

“…Primary pericardial synovial sarcoma is an extremely rare disorder (1). Because of its clinical rarity and wide histopathological diversity, its diagnosis may potentially be confused with various other pericardial diseases that show a pericardial mass with effusion on echocardiography and chest CT. Its most frequent presenting symptom is signs of cardiac tamponade; subsequent diagnostic imaging, including echocardiography or chest CT, reveals a pericardial mass requiring biopsy for diagnosis (2). Cytogenetic analysis is highly sensitive in the diagnosis and typically shows reciprocal chromosomal translocation (X;18 p11.2;q11.2) (3).…”