Synovial sarcomas that primarily arise from the heart and pericardium are extremely rare, especially the ones involving both sides of the heart. To date, few cases have been reviewed in the literature. Our patient was a 36-year-old man who presented with primary biatrial synovial sarcoma that also involved the tricuspid annulus, heart valves, and interatrial septum. The tumor was debulked to relieve the atrioventricular obstruction. Molecular analysis confirmed the diagnosis of synovial sarcoma with positive t (X;18) SYT-SSX gene fusion. The patient is currently receiving chemotherapy. From our review of the 20 cases thus far reported in the English-language medical literature, the tumor more frequently affects young male patients, and carries a poor prognosis. Early detection is difficult because of the aggressive nature of the tumor. Like other sarcomas, wide surgical resection remains the mainstay of therapy. Adjuvant radiation therapy for local recurrence and chemotherapy for control of systemic disease may have some beneficial effect on overall survival, but the benefit is likely limited.
[Show abstract][Hide abstract] ABSTRACT: We report the case of a 26-year-old man who experienced progressive left-sided chest pain and 2 episodes of near-syncope. Studies revealed a 15-cm mass in the upper left lung, a 10-cm mass in the medial base of the left lung, and a 5-cm left atrial mass that involved the left lung, infiltrated the left pulmonary vein, and prolapsed into the mitral valve, causing intermittent obstruction. The patient underwent surgical excision of the left atrial tumor. Pathologic evaluation confirmed the diagnosis of monophasic synovial sarcoma.To our knowledge, this is only the 3rd report of left atrial invasion and resultant mitral valve obstruction from a synovial sarcoma that infiltrated the pulmonary vein. We believe that this is the 1st documented case of a metastatic left atrial synovial sarcoma in monophasic form.
Texas Heart Institute journal / from the Texas Heart Institute of St. Luke's Episcopal Hospital, Texas Children's Hospital 01/2010; 37(4):472-5. · 0.65 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Primary cardiac synovial sarcoma is a rare disease. A 51-year-old man visited our hospital with the chief complaint of palpitations and shortness of breath while exercising. Copious bloody pericardial effusion and a multicystic intrapericardial tumor were detected. A primary cardiac malignant tumor was suspected, an open-chest tumor resection was performed with the objectives of diagnosis and treatment. Histologically, the tumor cells were uniformly spindle-shaped with an ovoid or oval nucleus, they had proliferated in fascicular fashion. In addition myxoid degeneration, a hemangiopericytomatous vascular pattern and pseudorosette formation were seen in some areas of the tumor. Based on the histopathological and immunohistochemical findings and reverse transcription polymerase chain reaction detection of SS18-SSX1 fusion transcripts, a monophasic fibrous type synovial sarcoma was diagnosed. Postoperative radiation therapy was administered and there had been no recurrence 9 months after the surgery.
Pathology International 03/2011; 61(3):150-5. DOI:10.1111/j.1440-1827.2010.02631.x · 1.69 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Primary sarcomas of the aorta, pulmonary artery, superior vena cava, inferior vena cava, and the heart are rare neoplasms. Aortic sarcomas are broadly categorized as either primarily luminal or primarily mural, with luminal sarcomas more likely to be misdiagnosed as thrombus. Pulmonary artery sarcomas are often mistaken for pulmonary embolism both clinically and at imaging. Vena caval sarcomas appear as intraluminal or extraluminal masses connecting to or filling the veins. The most common are leiomyosarcomas of the inferior vena cava. Primary sarcomas of the heart are rare and usually appear as heterogeneous aggressive masses.
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