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Year : 2015  |  Volume : 11  |  Issue : 3  |  Page : 659

Synovial sarcoma of the heart: A case report and literature review

Department of Medical Oncology, Rajiv Gandhi Government General Hospital and Madras Medical College, Chennai, Tamil Nadu, India

Date of Web Publication9-Oct-2015

Correspondence Address:
Prasad Eswaran
Department of Medical Oncology, Rajiv Gandhi Government General Hospital and Madras Medical College, Chennai, Tamil Nadu
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0973-1482.139391

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 > Abstract 

Primary cardiac tumors are of rare presentation. We present a case of synovial sarcoma of the right atrium treated in our institution. An initial diagnosis of right atrial myxoma was made based on clinico-radiological features. Intra-operatively, an irregular mass was found. Histopathologically, it was reported as monophasic synovial sarcoma. Immunohistochemistry was positive for S-100, B-cell lymphoma-2, MIC-2 and calretinin. Patient received adjuvant chemotherapy and is currently free of disease for 2 years and on regular follow-up.

Keywords: Cardiac sarcoma, cardiac tumors, monophasic synovial sarcoma

How to cite this article:
Eswaran P, Devadoss P, Narasimhan LS, Kannan K. Synovial sarcoma of the heart: A case report and literature review. J Can Res Ther 2015;11:659

How to cite this URL:
Eswaran P, Devadoss P, Narasimhan LS, Kannan K. Synovial sarcoma of the heart: A case report and literature review. J Can Res Ther [serial online] 2015 [cited 2020 May 28];11:659. Available from: http://www.cancerjournal.net/text.asp?2015/11/3/659/139391

 > Introduction Top

Primary cardiac tumors constitute a rare entity with autopsy incidence of about 0.001-0.28% compared to metastatic involvement of heart with a frequency of 1.5-21%. [1],[2],[3] There is no sex predilection and can arise in any part of the heart. [4] Most common histologies are angiosarcomas (37%), undifferentiated (24%), malignant fibrous histiocytoma (11-24%), leiomyosarcoma (9%) and osteosarcoma (3-9%). [5] Tumors reported as undifferentiated type are more commonly composed of more than mixed histologies showing chondro and/or rhabdomyosarcomatous differentiation proven by modern immunohistochemistry (IHC). [6] Synovial sarcoma of the heart is a rare presentation with rare incidence (<0.1%) reported in literature. [7],[8] We present a case of primary cardiac synovial sarcoma with clinical, pathological and immunohistochemical features treated at our institution.

 > Case report Top

The present case report is about a 35-year-old male patient who presented with complaints of chest pain and palpitations. His medical history was not notable for any other disease. Clinical examination was unremarkable. All hematological, renal, hepatic and coagulation parameters were normal. Echocardiography revealed a mass of size 6.3 cm × 3.3 cm arising from the septal leaflet of the tricuspid valve extending into the right ventricle and right ventricular outflow tract during diastole. Mild tricuspid regurgitation and paradoxical movement of inter ventricular septum and posterior wall of the left ventricle was also present. A clinical diagnosis of right atrial mass was made and planned for surgical removal. He underwent median sternotomy and surgical removal of the mass lesion. Per-operatively, he was found to have an irregular clustered tumor and was excised [Figure 1]. Post-operative recovery was uneventful.
Figure 1: Gross specimen

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 > Pathology Top

Histopathological section of the specimen revealed a tumor composed of sheets of cells with ovoid spindle shaped nuclei and wavy eosinophilic cytoplasm. Cells showed mild to moderate pleomorphism with areas of necrosis and hyalinization. IHC features were focally positive for S-100 p, positive for vimentin, B-cell lymphoma-2, CD99 and calretinin. CD34, desmin and pan cytokeratin (CK) were negative [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8] and [Figure 9]. The final pathological diagnosis was monophasic synovial sarcoma. Molecular analysis could not be completed in our case due to fixative technique.
Figure 2: H and E stain

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Figure 3: B-cell lymphoma-2

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Figure 4: Calretinin

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Figure 5: CD34

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Figure 6: CD99

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Figure 7: Cytokeratin

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Figure 8: Desmin

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Figure 9: S-100 p

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 > Adjuvant therapy Top

With the pathologic diagnosis of malignancy, a complete search for primary/other metastatic deposits was done with computed tomography (CT) scan. CT evaluation was negative for any primary tumor or metastases elsewhere. It was concluded to be a primary cardiac synovial sarcoma and adjuvant chemotherapy (CT) was started with adriamycin and ifosfamide. He received six cycles of adjuvant CT. Adjuvant radiation therapy (RT) was not planned. At present, patient is alive 2 years after completion of CT and is on regular follow-up without any signs of recurrence.

 > Discussion Top

Over 70-75% of the cardiac tumors are histopathologically benign myxomas most commonly arising from atria and the rest of them constitute malignant tumors of the heart of which synovial sarcoma is a rare entity. Most synovial sarcomas arise from right atrium, but there are reports of tumors originating from other sites also. [9],[10] In this case, tumor originates from septal leaflet of the tricuspid valve with ventricular extension [Table 1]. Majority of the cases had male preponderance and presented in 4 th and 5 th decade of life.
Table 1: Cases reported in literature primarily involving cardiac chamber[11] (tumors involving pericardium is excluded)

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Atrial tumors are more common and most of them are benign myxomas. Incidentally, malignant tumors are also found and resected. Echocardiography may be an initial useful tool for evaluation. However in our case, pre-operative echocardiography findings were more suggestive of myxoma. Cardiac magnetic resonance imaging (MRI) may be a useful modality in evaluation of cardiac masses in view of providing superior soft-tissue contrast resolution on extension into myocardium. [20] We recommend cardiac MRI for assessment of cardiac masses.

We have enlisted a table of IHC findings for malignant cardiac tumors [Table 2]. IHC confirmed the diagnosis of synovial sarcoma, although CK was negative and calretinin was positive in our case. This was proved in a study by Miettinen et al. [30] The definitive feature of synovial sarcoma is the presence of t (X: 18), which could not be completed in our case.
Table 2: IHC findings in malignant cardiac tumors

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There is no consensus regarding the adjuvant modality to be used after primary surgical resection. Complete surgical resection may not be feasible in many cases due to its location. Most case reports have indicated the use of adjuvant CT but there is no standard regime, although adriamycin based CT is commonly used. Role of RT is presently not clear.

 > Conclusion Top

Primary synovial sarcoma of the heart is a rare presentation. Cardiac imaging preferably by MRI should be done in evaluation of all cardiac masses. IHC and molecular analyses play an important role in the pathological confirmation of this tumor. Surgical resection is the primary modality. No consensus is available regarding adjuvant management.

 > References Top

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Centofanti P, Di Rosa E, Deorsola L, Dato GM, Patanè F, La Torre M, et al. Primary cardiac tumors: Early and late results of surgical treatment in 91 patients. Ann Thorac Surg 1999;68:1236-41.  Back to cited text no. 2
Silverman NA. Primary cardiac tumors. Ann Surg 1980;191:127-38.  Back to cited text no. 3
Donsbeck AV, Ranchere D, Coindre JM, Le Gall F, Cordier JF, Loire R. Primary cardiac sarcomas: An immunohistochemical and grading study with long-term follow-up of 24 cases. Histopathology 1999;34:295-304.  Back to cited text no. 4
Burke A, Virmani R, for the Armed Forces Institute of Pathology (USA), Universities Associated for Research and Education in Pathology. Neoplasms of the Heart and Great Vessels. Washington, DC: Armed Forces Institute of Pathology; 1996. p. 231.  Back to cited text no. 5
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Nicholson AG, Rigby M, Lincoln C, Meller S, Fisher C. Synovial sarcoma of the heart. Histopathology 1997;30:349-52.  Back to cited text no. 9
McGilbray TT, Schulz TK. Clinical picture: Primary cardiac synovial sarcoma. Lancet Oncol 2003;4:283.  Back to cited text no. 10
Hazelbag HM, Szuhai K, Tanke HJ, Rosenberg C, Hogendoorn PC. Primary synovial sarcoma of the heart: A cytogenetic and molecular genetic analysis combining RT-PCR and COBRA-FISH of a case with a complex karyotype. Mod Pathol 2004;17:1434-9.  Back to cited text no. 11
McAllister HA, Fenoglio JJ. Malignant tumors of the heart and pericardium. In: Tumors of the Cardiovas-cular System (fasc. 15). AFIP. Atlas of Tumor Pathology. 2 nd Series. Bethesda: AFIP; 1978. p. 108.  Back to cited text no. 12
Sheffield EA, Corrin B, Addis BJ, Gelder C. Synovial sarcoma of the heart arising from a so-called mesothelioma of the atrio-ventricular node. Histopathology 1988;12:191-201.  Back to cited text no. 13
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;99:567-8.  Back to cited text no. 14
Burke AP, Cowan D, Virmani R. Primary sarcomas of the heart. Cancer 1992;69:387-95.  Back to cited text no. 15
Karn CM, Socinski MA, Fletcher JA, Corson JM, Craighead JE. Cardiac synovial sarcoma with translocation (X; 18) associated with asbestos exposure. Cancer 1994;73:74-8.  Back to cited text no. 16
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Casselman FP, Gillinov AM, Kasirajan V, Ratliff NB, Cosgrove DM 3 rd . Primary synovial sarcoma of the left heart. Ann Thorac Surg 1999;68:2329-31.  Back to cited text no. 18
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Constantinou LL, Charitos CE, Lariou CM, Garyphalos DJ, Douras AL, Vichas GJ, et al. Primary synovial cardiac sarcoma: A rare cause of tamponade. Eur Heart J 1996;17:1766-8.  Back to cited text no. 21
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Zhao Q, Geha AS, Devries SR, Tshibaka C, Wu SJ, Lavi N, et al. Biatrial primary synovial sarcoma of the heart. J Am Soc Echocardiogr 2007;20:197.e1-4.  Back to cited text no. 24
Sakai M, Takami H, Joyama S, Tomita Y, Yamamoto T, Araki N, et al. Cardiac synovial sarcoma swinging through the aortic valve. Ann Thorac Surg 2011;92:1129.  Back to cited text no. 25
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Miettinen M, Limon J, Niezabitowski A, Lasota J. Calretinin and other mesothelioma markers in synovial sarcoma: Analysis of antigenic similarities and differences with malignant mesothelioma. Am J Surg Pathol 2001;25:610-7.  Back to cited text no. 30


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9]

  [Table 1], [Table 2]


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