Case Study
Primary cardiac synovial sarcoma Imran Khan, Saira Gul, Zafar Tufail, Kamran Khan, Praman Sharma and Abdul Waheed
Asian Cardiovascular & Thoracic Annals 2015, Vol. 23(6) 713–715 ß The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0218492314524753 aan.sagepub.com
Abstract Approximately 10% of soft tissue sarcomas are synovial sarcomas, and 90% of these occur in the extremities. Among the primary tumors in the heart, 25% are malignant. Primary synovial sarcoma of the heart is an extremely rare entity. A myriad of investigations such as histopathology, immunohistochemistry, electron microscopy, and molecular genetic techniques are required for confirmation of the diagnosis. The tumor is nearly always lethal, but surgical resection with chemotherapy may prolong the life of the patient. We describe the case of a young patient with a primary synovial sarcoma arising from the right ventricle.
Keywords Adolescent, heart neoplasms, heart ventricles, sarcoma, synovial
Introduction Primary cardiac synovial sarcoma is extremely rare.1 It is an aggressive tumor of the heart and may occur in any chamber but has a predilection for the right heart. The available data suggest it is more common in young males. Because of the scant experience with this tumor, no consensus regarding the treatment of choice has been reached. Complete resection along with chemotherapy has been shown to be of some benefit. We are reporting a case of primary sarcoma of the heart from the Punjab Institute of Cardiology, Lahore, Pakistan. To the best of our knowledge, this is the first reported case of intracardiac synovial sarcoma in the Pakistani population.
Case report A 17-year-old boy presented to our outpatient department with a 1-month history of anorexia and a 3-day history of shortness of breath. He also complained of heaviness and tightness in the chest on exertion. He had engorged neck veins and facial swelling. An electrocardiogram showed nothing remarkable. Echocardiography revealed a dilated right atrium and right ventricle, with a large heterogeneous mass (40 70 mm) attached to the right ventricular side of the interventricular septum, occupying almost the
whole right ventricle and prolapsing across the pulmonary and tricuspid valves during systole. We planned a cardiac computed tomography scan and transesophageal echocardiogram, but the patient became hemodynamically unstable, thus early surgical intervention was undertaken. The surgery was performed through a median sternotomy approach with cardiopulmonary bypass using aortic and double-venous cannulation. The right ventricle was approached through the right atrium. A huge mass was observed arising from the interventricular septum and involving the septal leaflet of the tricuspid valve, extending to the pulmonary artery on the right side (Figure 1). Owing to the extensive nature of the tumor, a limited resection was planned. The septal and posterior leaflets of the tricuspid valve were cleared of tumor as much as possible. An 8.0 7.0 6.5-cm mass could be resected without damage to the surrounding structures. The cut surface was firm, grey-white, and myxoid. The patient remained stable hemodynamically in the immediate
Department of Cardiac Surgery, Punjab Institute of Cardiology, Lahore, Pakistan Corresponding author: Imran Khan, Room No. B-20, Old Doctor Hostel, Punjab Institute of Cardiology, Jail Road, Lahore, Pakistan. Email: [email protected]
Downloaded from aan.sagepub.com at UNIV OF LETHBRIDGE on November 14, 2015
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Asian Cardiovascular & Thoracic Annals 23(6)
Figure 1. The tumor can be seen as a bluish grey mass through the right atriotomy. Figure 3. Cytokeratin staining. Original magnification 10.
Figure 2. Epithelial membrane antigen. Original magnification 10.
postoperative period. Histopathology showed a tumor composed of hypocellular and hypercellular areas. There were scattered fascicles of spindle cells showing eosinophilic cytoplasm and round to oval nuclei. Mild nuclear pleomorphism and a few scattered mitosis with atypical mitosis were also seen. The tumor had areas of necrosis. Immunohistochemical staining showed that the tumor was positive for epithelial membrane antigen (Figure 2), CD34, and cytokeratin (Figure 3). A diagnosis of synovial sarcoma was made. The patient was investigated with computed tomography of the brain, chest, and abdomen, as well as a bone scan and ancillary investigations of the primary site. Upon finding no primary, the patient was diagnosed to have a primary synovial sarcoma of the heart. He was discharged with marked symptomatic improvement, and referred to an oncologist for chemotherapy. He successfully completed the chemotherapy course, and after 11 months of follow-up, he was alive and asymptomatic.
Discussion Approximately 25% primary tumors of the heart are malignant.2 Sarcomas are the most common malignant
tumors and include myxosarcoma, liposarcoma, angiosarcoma, fibrosarcoma, leiomyosarcoma, osteosarcoma, synovial sarcoma, rhabdomyosarcoma, neurofibrosarcoma, malignant fibrous histiocytoma, and undifferentiated sarcoma. The term synovial sarcoma continues to be the designation of choice, but it is a misnomer. It is considered to be a tumor of unknown histogenesis because the presence of epithelial markers such as cytokeratin is not a feature of normal synovium.3 The clinical manifestations include shortness of breath, chest pain, systemic or constitutional symptoms, and sometimes, patients are asymptomatic. Presentation with transient ischemic attacks and facial and neck vein congestion due to a mass effect has been reported. Echocardiography, cardiac computed tomography, and magnetic resonance imaging are good investigations for diagnosis of a mass in the heart. Preoperative diagnosis of a sarcoma can be a challenge because the presentation can be just like any other mass. Policarpio-Nicolas and colleagues4 reported the preoperative diagnosis of a primary sarcoma of the heart using endoscopic ultrasound-guided fine-needle aspiration. How much this approach may be applicable in clinical settings needs further evaluation. The tumor is located primarily in the right heart, but location in the left side has also been reported.5 Few cases have been reported in the right ventricle. The tumor can also arise primarily from the vessels, although only 6 such cases have been reported.6 Of the previously reported cases, 51.5% could be resected partially, while 33.3% underwent complete resection. In our case, the tumor could be resected only partially. Two histologic patterns have been described: a monophasic type, and a biphasic type. Biphasic is considered the classic type and is generally recognizable by the coexistence of morphologically different but histogenetically related epithelial cells and fibroblast-like spindle cells. Only the poorly differentiated variants
Downloaded from aan.sagepub.com at UNIV OF LETHBRIDGE on November 14, 2015
Khan et al.
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show a mitotic count of more than 2 mitotic figures per high-power field. The monophasic variants show positive immunostaining of the spindle cells for keratin and epithelial membrane antigen. The synovial sarcoma in our case was of the monophasic variety, with areas of poor differentiation as revealed by histopathology and immunohistochemical staining. Surgical resection is the mainstay of treatment, but it may not always be possible. Chemotherapy with cytotoxic drugs has been shown to be of some promise in synovial sarcomas. Some studies have shown a response rate of 58%.7 How effective this therapy is in primary sarcomas of the heart needs to be proved. Median survival has been reported to be 27 months with chemotherapy and 5.5 months in patients not receiving chemotherapy.8 Primary sarcomas of the heart have a consistent genetic makeup of t(X;18) which leads to a fusion protein SYT/SSX. This consistent behavior may be utilized for molecular targeting. Because of the limited number of cases, there is little experience with the treatment options. A right-sided mass in a young adolescent should raise the suspicion of a malignant mass in the heart, as data from the literature suggest. Funding This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.
Conflict of interest statement None declared.
References 1. Lv X, Guo X, Chen X, et al. Primary cardiac synovial sarcoma. J Card Surg 2010; 25: 288–290. 2. Paraskevaidis IA, Michalakeas CA, Papadopoulos CH and Anastasiou-Nana M. Cardiac tumors. ISRN Oncol 2011; 2011: 208929. 3. Weiss SW and Goldblum JR. Malignant soft tissue tumors of uncertain type. Soft Tissue Tumors, 5th ed. Philadelphia, PA: Mosby Elsevier, 2008, pp. 1161–1182. 4. Policarpio-Nicolas ML, Alasadi R, Nayar R and De Frias DV. Synovial sarcoma of the heart: report of a case with diagnosis by endoscopic ultrasound-guided fine needle aspiration biopsy. Acta Cytol 2006; 50: 683–686. 5. Fujioka M, Suehiro S, Shibata T, Kinoshita H, Wakasa K and Haba T. Primary cardiac synovial sarcoma—a case report. Jpn J Thorac Cardiovasc Surg 1998; 46: 923–927. 6. Wise KB, Said SM, Clark CJ, et al. Resection of a giant primary synovial sarcoma of the inferior vena cava extending into the right atrium with caval reconstruction under cardiopulmonary bypass and circulatory arrest. Perspect Vasc Surg Endovasc Ther 2012; 24: 95–101. 7. Adjuvant chemotherapy for localised resectable soft-tissue sarcoma of adults: meta-analysis of individual data. Sarcoma Meta-Analysis Collaboration. Lancet 1997; 350: 1647–1654. 8. Wang JG and Li NN. Primary cardiac synovial sarcoma. Ann Thorac Surg 2013; 95: 2202–2209.
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Primary cardiac synovial sarcoma Imran Khan, Saira Gul, Zafar Tufail, Kamran Khan, Praman Sharma and Abdul Waheed
Asian Cardiovascular & Thoracic Annals 2015, Vol. 23(6) 713–715 ß The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0218492314524753 aan.sagepub.com
Abstract Approximately 10% of soft tissue sarcomas are synovial sarcomas, and 90% of these occur in the extremities. Among the primary tumors in the heart, 25% are malignant. Primary synovial sarcoma of the heart is an extremely rare entity. A myriad of investigations such as histopathology, immunohistochemistry, electron microscopy, and molecular genetic techniques are required for confirmation of the diagnosis. The tumor is nearly always lethal, but surgical resection with chemotherapy may prolong the life of the patient. We describe the case of a young patient with a primary synovial sarcoma arising from the right ventricle.
Keywords Adolescent, heart neoplasms, heart ventricles, sarcoma, synovial
Introduction Primary cardiac synovial sarcoma is extremely rare.1 It is an aggressive tumor of the heart and may occur in any chamber but has a predilection for the right heart. The available data suggest it is more common in young males. Because of the scant experience with this tumor, no consensus regarding the treatment of choice has been reached. Complete resection along with chemotherapy has been shown to be of some benefit. We are reporting a case of primary sarcoma of the heart from the Punjab Institute of Cardiology, Lahore, Pakistan. To the best of our knowledge, this is the first reported case of intracardiac synovial sarcoma in the Pakistani population.
Case report A 17-year-old boy presented to our outpatient department with a 1-month history of anorexia and a 3-day history of shortness of breath. He also complained of heaviness and tightness in the chest on exertion. He had engorged neck veins and facial swelling. An electrocardiogram showed nothing remarkable. Echocardiography revealed a dilated right atrium and right ventricle, with a large heterogeneous mass (40 70 mm) attached to the right ventricular side of the interventricular septum, occupying almost the
whole right ventricle and prolapsing across the pulmonary and tricuspid valves during systole. We planned a cardiac computed tomography scan and transesophageal echocardiogram, but the patient became hemodynamically unstable, thus early surgical intervention was undertaken. The surgery was performed through a median sternotomy approach with cardiopulmonary bypass using aortic and double-venous cannulation. The right ventricle was approached through the right atrium. A huge mass was observed arising from the interventricular septum and involving the septal leaflet of the tricuspid valve, extending to the pulmonary artery on the right side (Figure 1). Owing to the extensive nature of the tumor, a limited resection was planned. The septal and posterior leaflets of the tricuspid valve were cleared of tumor as much as possible. An 8.0 7.0 6.5-cm mass could be resected without damage to the surrounding structures. The cut surface was firm, grey-white, and myxoid. The patient remained stable hemodynamically in the immediate
Department of Cardiac Surgery, Punjab Institute of Cardiology, Lahore, Pakistan Corresponding author: Imran Khan, Room No. B-20, Old Doctor Hostel, Punjab Institute of Cardiology, Jail Road, Lahore, Pakistan. Email: [email protected]
Downloaded from aan.sagepub.com at UNIV OF LETHBRIDGE on November 14, 2015
714
Asian Cardiovascular & Thoracic Annals 23(6)
Figure 1. The tumor can be seen as a bluish grey mass through the right atriotomy. Figure 3. Cytokeratin staining. Original magnification 10.
Figure 2. Epithelial membrane antigen. Original magnification 10.
postoperative period. Histopathology showed a tumor composed of hypocellular and hypercellular areas. There were scattered fascicles of spindle cells showing eosinophilic cytoplasm and round to oval nuclei. Mild nuclear pleomorphism and a few scattered mitosis with atypical mitosis were also seen. The tumor had areas of necrosis. Immunohistochemical staining showed that the tumor was positive for epithelial membrane antigen (Figure 2), CD34, and cytokeratin (Figure 3). A diagnosis of synovial sarcoma was made. The patient was investigated with computed tomography of the brain, chest, and abdomen, as well as a bone scan and ancillary investigations of the primary site. Upon finding no primary, the patient was diagnosed to have a primary synovial sarcoma of the heart. He was discharged with marked symptomatic improvement, and referred to an oncologist for chemotherapy. He successfully completed the chemotherapy course, and after 11 months of follow-up, he was alive and asymptomatic.
Discussion Approximately 25% primary tumors of the heart are malignant.2 Sarcomas are the most common malignant
tumors and include myxosarcoma, liposarcoma, angiosarcoma, fibrosarcoma, leiomyosarcoma, osteosarcoma, synovial sarcoma, rhabdomyosarcoma, neurofibrosarcoma, malignant fibrous histiocytoma, and undifferentiated sarcoma. The term synovial sarcoma continues to be the designation of choice, but it is a misnomer. It is considered to be a tumor of unknown histogenesis because the presence of epithelial markers such as cytokeratin is not a feature of normal synovium.3 The clinical manifestations include shortness of breath, chest pain, systemic or constitutional symptoms, and sometimes, patients are asymptomatic. Presentation with transient ischemic attacks and facial and neck vein congestion due to a mass effect has been reported. Echocardiography, cardiac computed tomography, and magnetic resonance imaging are good investigations for diagnosis of a mass in the heart. Preoperative diagnosis of a sarcoma can be a challenge because the presentation can be just like any other mass. Policarpio-Nicolas and colleagues4 reported the preoperative diagnosis of a primary sarcoma of the heart using endoscopic ultrasound-guided fine-needle aspiration. How much this approach may be applicable in clinical settings needs further evaluation. The tumor is located primarily in the right heart, but location in the left side has also been reported.5 Few cases have been reported in the right ventricle. The tumor can also arise primarily from the vessels, although only 6 such cases have been reported.6 Of the previously reported cases, 51.5% could be resected partially, while 33.3% underwent complete resection. In our case, the tumor could be resected only partially. Two histologic patterns have been described: a monophasic type, and a biphasic type. Biphasic is considered the classic type and is generally recognizable by the coexistence of morphologically different but histogenetically related epithelial cells and fibroblast-like spindle cells. Only the poorly differentiated variants
Downloaded from aan.sagepub.com at UNIV OF LETHBRIDGE on November 14, 2015
Khan et al.
715
show a mitotic count of more than 2 mitotic figures per high-power field. The monophasic variants show positive immunostaining of the spindle cells for keratin and epithelial membrane antigen. The synovial sarcoma in our case was of the monophasic variety, with areas of poor differentiation as revealed by histopathology and immunohistochemical staining. Surgical resection is the mainstay of treatment, but it may not always be possible. Chemotherapy with cytotoxic drugs has been shown to be of some promise in synovial sarcomas. Some studies have shown a response rate of 58%.7 How effective this therapy is in primary sarcomas of the heart needs to be proved. Median survival has been reported to be 27 months with chemotherapy and 5.5 months in patients not receiving chemotherapy.8 Primary sarcomas of the heart have a consistent genetic makeup of t(X;18) which leads to a fusion protein SYT/SSX. This consistent behavior may be utilized for molecular targeting. Because of the limited number of cases, there is little experience with the treatment options. A right-sided mass in a young adolescent should raise the suspicion of a malignant mass in the heart, as data from the literature suggest. Funding This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.
Conflict of interest statement None declared.
References 1. Lv X, Guo X, Chen X, et al. Primary cardiac synovial sarcoma. J Card Surg 2010; 25: 288–290. 2. Paraskevaidis IA, Michalakeas CA, Papadopoulos CH and Anastasiou-Nana M. Cardiac tumors. ISRN Oncol 2011; 2011: 208929. 3. Weiss SW and Goldblum JR. Malignant soft tissue tumors of uncertain type. Soft Tissue Tumors, 5th ed. Philadelphia, PA: Mosby Elsevier, 2008, pp. 1161–1182. 4. Policarpio-Nicolas ML, Alasadi R, Nayar R and De Frias DV. Synovial sarcoma of the heart: report of a case with diagnosis by endoscopic ultrasound-guided fine needle aspiration biopsy. Acta Cytol 2006; 50: 683–686. 5. Fujioka M, Suehiro S, Shibata T, Kinoshita H, Wakasa K and Haba T. Primary cardiac synovial sarcoma—a case report. Jpn J Thorac Cardiovasc Surg 1998; 46: 923–927. 6. Wise KB, Said SM, Clark CJ, et al. Resection of a giant primary synovial sarcoma of the inferior vena cava extending into the right atrium with caval reconstruction under cardiopulmonary bypass and circulatory arrest. Perspect Vasc Surg Endovasc Ther 2012; 24: 95–101. 7. Adjuvant chemotherapy for localised resectable soft-tissue sarcoma of adults: meta-analysis of individual data. Sarcoma Meta-Analysis Collaboration. Lancet 1997; 350: 1647–1654. 8. Wang JG and Li NN. Primary cardiac synovial sarcoma. Ann Thorac Surg 2013; 95: 2202–2209.
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