|Year : 2015 | Volume
| Issue : 3 | Page : 659
Synovial sarcoma of the heart: A case report and literature review
Prasad Eswaran MBBS, MD, DMRT , Premkumar Devadoss, Lakshmi S Narasimhan, Kalaichelvi Kannan
Department of Medical Oncology, Rajiv Gandhi Government General Hospital and Madras Medical College, Chennai, Tamil Nadu, India
|Date of Web Publication||9-Oct-2015|
Department of Medical Oncology, Rajiv Gandhi Government General Hospital and Madras Medical College, Chennai, Tamil Nadu
Source of Support: None, Conflict of Interest: None
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
| > Introduction|| |
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%. ,, There is no sex predilection and can arise in any part of the heart.  Most common histologies are angiosarcomas (37%), undifferentiated (24%), malignant fibrous histiocytoma (11-24%), leiomyosarcoma (9%) and osteosarcoma (3-9%).  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).  Synovial sarcoma of the heart is a rare presentation with rare incidence (<0.1%) reported in literature. , We present a case of primary cardiac synovial sarcoma with clinical, pathological and immunohistochemical features treated at our institution.
| > Case report|| |
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.
| > Pathology|| |
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.
| > Adjuvant therapy|| |
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|| |
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. , 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 (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.  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.  The definitive feature of synovial sarcoma is the presence of t (X: 18), which could not be completed in our case.
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|| |
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.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9]
[Table 1], [Table 2]