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| Year : 2012 | Volume
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| Issue : 2 | Page : 227-229 |
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| Pericardium: An exceedingly rare site for a primary biphasic synovial sarcoma |
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Sarathchandra Kodikara
Department of Forensic Medicine, Faculty of Medicine, University of Peradeniya, Sri Lanka
Click here for correspondence address and email
| Date of Web Publication | 3-Jul-2012 |
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 Abstract | | |
Pericardial neoplasms are rare. Among pericardial neoplasms, metastatic spread is more common and primary synovial sarcomas of the pericardium are exceedingly rare. A 61-year-old man was found dead in bed. Autopsy revealed the cause of death as atherosclerotic and hypertensive heart disease. There was a pericardial soft tissue mass lying predominantly over the left atrium. The cut surface was bulging out and grayish white in color with cystic changes. Microscopy showed spindle cells in densely cellular fascicles surrounding the epithelial cells. The epithelial cells were cuboidal and formed glandular structures and cleft-like spaces. Immunohistochemistry demonstrated cytokeratin and vimentin positivity in the spindled areas. Molecular analysis confirmed the diagnosis. Synovial sarcoma may be mistaken for other common neoplasms occurring in the pericardium due to its rarity. In all cases of pericardial tumors, a diligent search for synovial sarcoma by way of histological analysis including, immunohistochemistry and molecular analysis is indicated. Keywords: Biphasic, pericardium, primary synovial sarcoma
How to cite this article:
Kodikara S. Pericardium: An exceedingly rare site for a primary biphasic synovial sarcoma. Indian J Pathol Microbiol 2012;55:227-9 |
| Introduction | |  |
Cardiac and pericardial neoplasms are rare. Among pericardial neoplasms, metastatic spread is more common than primary pericardial neoplasms. [1] In terms of primary pericardial neoplasms, mesothelioma is the most common. [2] Other primary pericardial neoplasms include malignant spindle cell tumors, pericardial cysts, liposarcomas, lipomas, and teratomas. Primary synovial sarcomas of the pericardium are exceedingly rare. [3] Review of the literature revealed only a very few reported cases in this regard. [3],[4]
| Case Report | | |
A 61-year-old man was found dead in the bed. He had a past history of hypertension and was not on regular treatment. There was no family history of malignancy.
At autopsy upon opening the chest cavity, there was a 5.0 × 3.0 × 2.5 cm, irregular soft tissue mass (93 g) in the stretched out and tensed pericardium anteriorly lying predominantly over the left atrium. The cut surface was bulging out and grayish white in color with cystic changes [Figure 1]. The surrounding tissues were not compressed by this mass. There was moderate pericardial effusion. The tumor was not metastatic, and there was no evidence of any other pericardial tumor identified. | Figure 1: The cut surface of the primary biphasic pericardial synovial sarcoma
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The heart was (530 g) enlarged with left-sided chamber dilation and prominent left ventricular hypertrophy, while the myocardium showed multiple focal fibrotic patches. The epicardial coronary arteries had a right dominant circulation, and showed moderate to severe atherosclerotic luminal stenosis involving the left anterior descending (up to 90%), circumflex (up to 70%), and right (up to 80%) coronary arteries. The subcapsular renal cortex possessed a mild to moderate granular texture.
Microscopy showed fibroblast-like spindle cells in densely cellular fascicles surrounding the epithelial cells. The epithelial cells were cuboidal and formed glandular structures and cleft-like spaces [Figure 2]. Immunohistochemistry demonstrated patchy cytokeratin and vimentin (Dako, Glostrup, and Denmark) positivity in the spindled areas but negative for all of the markers (calretinin, CK5/6, WT-1, and D2-40) commonly positive for malignant mesothelioma. Molecular analysis revealed a SYT-SSX1 fusion transcript indicative of a t(X; 18) translocation mutation. | Figure 2: Spindle cell infi ltrate and epithelial cell component with both solid and glandular architecture forming biphasic synovial sarcoma (hematoxylin and eosin, ×40)
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Microscopy of the heart showed cardiomyocyte hypertrophy associated with mild to moderate interstitial and perivascular fibrous tissue deposition. There were occasional subendocardial foci of reticular-type fibrous tissue deposition. No acute ischemic changes were identified and no myocardial inflammation was observed. The coronary arteries showed near occlusion of the lumen by fibroatheromatous and fibromuscular atherosclerosis. Recanalization of the vascular lumen of small muscularized arteries was noted with abundant hemosiderin granules. Both kidneys showed moderate nephrosclerosis.
The cause of death was concluded as atherosclerotic and hypertensive heart disease in a person with a primary biphasic pericardial synovial sarcoma.
| Discussion | | |
Primary cardiac tumors are rare and these are mostly benign atrial myxomas. [3] The majority of malignant cardiac tumors consist of sarcomas. [3] These include angiosarcoma, fibrosarcoma, rhabdomyosarcoma, osteosarcoma, malignant fibrous histiocytoma, leiomyosarcoma, myxosarcoma, neurofibrosarcoma, undifferentiated sarcoma, and synovial sarcoma.
Among pericardial neoplasms, metastatic spread from lung carcinoma, breast carcinoma, lymphoma, and leukemia are more common than primary pericardial neoplasms. [1] In terms of primary pericardial neoplasms, mesothelioma is the commonest while synovial sarcoma is extremely rare. [2] Pericardial mesotheliomas are usually associated with a prior history of exposure to asbestos and diffusely infiltrate the pericardium. Synovial sarcoma is usually a solitary mass, and not associated with a history of asbestos exposure. [3] Other pericardial tumors include malignant spindle cell tumors, pericardial cysts, liposarcomas, lipomas, and teratomas.
Synovial sarcoma is a soft tissue lesion derived from primitive mesenchymal cells, and commonly occurs in soft tissues associated with joint capsules, tendon sheaths, and bursae of extremities. It rarely occurs in other locations of the body such as head and neck, tonsils, skin, blood vessels, abdominal wall, prostate, nerves, pleural cavity, and kidney. [5],[6] Synovial sarcoma frequently arises from the late second through fourth decade. [5] This case shows that it could occur even in the seventh decade. Although this tumor has a very poor prognosis, it did not cause any complications and did not contribute to death in this case. [7]
Synovial sarcomas are histologically monophasic, biphasic, or poorly differentiated. [5] Monophasic variety has two types: (1) monophasic fibrous (spindle cell) type and (2) monophasic epithelial type. [5] The monophasic fibrous (spindle cell) type resembles and may be confused with fibrosarcoma, malignant peripheral nerve sheath tumors, and malignant hemangiopericytoma. Biphasic variety is composed of the admixture of fibroblast-like spindle cell and epithelial cell component, as in this case.
As the tumor cells yield positive reaction for keratin and epithelial membrane antigen, immunohistochemistry is useful in identifying them. [3],[8] Non-neoplastic and/or diseased synovium do not stain with antibodies to keratin. [8] In this case, immunostaining demonstrated cells weakly positive for cytokeratin and vimentin and supported the diagnosis.
Cloning of the breakpoints of chromosomal translocations has shown that these translocations result in the production of new chimeric transcripts that provide tumor-specific markers that can be detected by reverse transcriptase-polymerase chain reaction. [4] A chromosomal aberration has been detected in a majority of synovial sarcomas and this is characterized by t(X; 18) translocation mutation. [9] This translocation involves the SSX gene on the X chromosome (Xp 11) and the SYT gene on chromosome 18 (18q11). [3] There are two subtypes of SSX (SSX1 and SSX2) that have been found to be of independent prognostic significance. [10] In this case, strengthening the macroscopic and microscopic findings, the SYT-SSX1 fusion transcript indicative of a t(X; 18) translocation mutation, helped to arrive at the final diagnosis. Molecular diagnosis is paramount especially when the histological diagnosis is difficult.
Common sites of metastases are the mediastinum, lung, skeleton, and occasionally the regional lymph nodes. There was no evidence of synovial sarcoma elsewhere in this deceased.
Thus a rare case of primary biphasic synovial sarcoma is reported, and it confirms previous observations that it can also form in the pericardium. This is significant, because it may be mistaken for other common neoplasms occurring in the pericardium due to its rarity. On this basis, in all cases of pericardial tumors, a diligent search for synovial sarcoma by way of histological analysis including immunohistochemistry and molecular analysis is indicated.
| Acknowledgment | | |
The author would like to thank Drs. Sumith Ambepitiya, A. Dayapala and Ramesh Alagiyawanna for their helpful discussions. The author is also grateful to Mr. V. Sahadevan and Mr A.M. Amjad for technical assistance.
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Correspondence Address:
Sarathchandra Kodikara
Department of Forensic Medicine, Faculty of Medicine, University of Peradeniya
Sri Lanka
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/0377-4929.97884
[Figure 1], [Figure 2] |
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