Ann Thorac Surg 2000;69:1949-1951
© 2000 The Society of Thoracic Surgeons
Case reports
Primary right atrial synovial sarcoma manifesting as transient ischemic attacks
Bindu Bittira, MDa,
John Tsang, MDa,
Thao Huynh, MDa,
Jean-François Morin, MDa,
Istvan Hüttner, MD, PhDb
a Department of Cardiothoracic Surgery, McGill University Health Centre, Montreal, Quebec, Canada
b Department of Pathology, McGill University Health Centre, Montreal, Quebec, Canada
Address reprint requests to Dr Morin, Department of Cardiothoracic Surgery, McGill University Health Centre, Montreal General Hospital, 1650 Cedar Ave, Room L9513, Montreal, PQ, H3G 1A4 Canada
e-mail: j.f.morin{at}videotran.ca
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Abstract
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Primary tumors of the heart are rare and most of them benign. The majority of benign cardiac tumors are myxomas while almost all malignant cardiac tumors are sarcomas. We present a case of primary right atrial synovial sarcoma, a form of sarcoma particularly rare in the heart. The tumor manifested clinically as transient ischemic attacks probably related to a patent foramen ovale allowing paradoxical tumor embolization.
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Introduction
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Primary tumors of the heart are rare and most of them benign. The majority of benign cardiac tumors are myxomas, while almost all malignant cardiac tumors are sarcomas. In 1,000 surgically resected cardiac tumors from several series of the last 10 years, the proportion of cardiac sarcomas was only 10%, while the proportion of cardiac myxomas was 77% [1]. We present a case of primary right atrial synovial sarcoma, a form of sarcoma particularly rare in the heart [2]. The tumor manifested clinically as transient ischemic attacks probably related to a patent foramen ovale allowing paradoxical tumor embolization.
A 47-year-old man presented to the Emergency Room with a sudden onset of transient right arm weakness, right-sided facial droop, and aphasia. Previous history was positive only for smoking and a dry cough. Physical examination was normal except for an abnormal extra-systolic sound and an S4. Echocardiography revealed a 3.9 x 4.2 cm mass adherent to the atrial side of the septal tricuspid leaflet, thought to represent atrial myxoma. Computed tomography scan of the chest demonstrated, in addition, multiple well-defined pulmonary nodules in both lungs which were interpreted to represent embolization from the right atrial myxoma. In view of the possibility of further embolization, resection of the right atrial mass was decided.
An intraoperative transesophageal echocardiogram confirmed the presence of a large (4.5 cm) right atrial mass adherent to the tricuspid valve (Fig 1) with the presence of mild tricuspid regurgitation. The tumor was resected along with the central portion of the septal leaflet. The defect in the septal leaflet was repaired using the untreated pericardium, and a small patent foramen ovale was clearly identified and oversewn. After closing the atrium and de-airing the heart, biopsies of the lung nodules were performed. The patient was easily weaned off cardiopulmonary bypass and a transesophageal echocardiogram was performed, revealing complete resection of the right atrial mass. The patient had an uneventful postoperative course.
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Fig 1. View of intraoperative transesophageal echocardiogram showing large atrial mass adherent to the tricuspid valve.
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Histopathologic examination revealed that the right atrial mass was a biphasic malignant tumor with distinctive epithelial and spindle cell components, characteristic of synovial sarcoma. Immunohistochemistry demonstrated reactivity of the epithelial component with the epithelial markers cytokeratin (Fig 2A), AE-3, and EMA, and reactivity of the spindle cell component with the mesenchymal marker vimentin (Fig 2B) confirming the diagnosis. The pulmonary nodules were diagnosed as metastases originating from embolization of the primary right atrial synovial sarcoma.
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Fig 2. Immunohistochemical preparations of the right atrial mass showing characteristic features of biphasic synovial sarcoma: islands of gland forming cuboidal/columnar epithelial cells that are surrounded by fibrosarcoma-like spindle cell elements. (A) The epithelial component strongly reactive for the epithelial marker cytokeratin. (B) The spindle cell component is strongly reactive for the mesenchymal marker vimentin.
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The patient was referred to medical oncology and will undergo a course of adriamycin chemotherapy.
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Comment
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Synovial sarcoma occurs most frequently in the para-articular regions of the extremities and arises from the primitive arthrogenous mesenchyme with the capacity to differentiate toward both mesenchymal and epithelial cell lines [3]. However, it is encountered in areas without any relationship to synovial structures such as the tongue, parapharyngeal region, or abdomen [3], and very rarely also arises from blood vessels [4], and from the heart [2, 5]. In the English literature, 6 cases of primary synovial sarcoma of the heart were documented up to 1997 [2]. Therefore, our report represents the 7th case of this rare malignant tumor arising in the heart.
The clinical signs and symptoms for right atrial tumors are usually nonspecific. While supraventricular and ventricular arrhythmias are common, cardiac symptoms in patients with primary tumors of the heart may not always be present. The unusual clinical manifestation of transient ischemic attacks in our case resulted from embolization of the right atrial tumor through the patent foramen ovale identified during surgery or, less likely, it was related to hypercoagulability.
Retrospectively, the location of the tumor mass on the right side of the heart could have raised suspicion of a malignant tumor, as cardiac myxomas occur preferentially in the left atrium [1]. The pulmonary nodules could have further raised suspicion of metastases originating from a malignant tumor, although embolization from a rare right atrial myxoma also may result in a similar radiological picture. Unfortunately, a better diagnostic approach to early diagnosis may not improve the overall survival because of the usual early dissemination of cardiac sarcomas [6]. So far, surgical resection or heart transplantation remains the only hope for palliation [5].
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References
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Burke A, Virmani R. Tumors of the heart and great vessels. In: Burke A, Virmani R, eds. Atlas of tumor pathology, Third series, Fascicle 16. Washington, DC: Armed Forces Institute of Pathology, 1996:1–11.
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Nicholson A.G., Rigby M., Lincoln C., Meller S., Fisher C. Synovial sarcoma of the heart. Histopathology 1997;30:349-352.[Medline]
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Enzinger FM, Weiss SW. Synovial sarcoma. In: Enzinger FM, Weiss SW, eds. Soft tissue tumors, 3rd ed. St. Louis: Mosby-Year Book, 1995:757–86.
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Miettinen M., Santavirta S., Slätis P. Intravascular synovial sarcoma. Hum Pathol 1987;18:1075-1077.[Medline]
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Siebenmann R., Jenni R., Makek M., Oelz O., Turina M. Primary synovial sarcoma of the heart treated by heart transplantation. J Thorac Cardiovasc Surg 1990;99:567-568.[Medline]
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Hammoudeh A.J., Chaaban F., Watson R.M., Millman A. Transesophageal echocardiography-guided transvenous endomyocardial biopsy used to diagnose primary cardiac angiosarcoma. Cathet Cardiovasc Diagn 1996;37:347-349.[Medline]
Accepted for publication November 20, 1999.
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Abstract
Introduction
