- Email: [email protected]
Synovial sarcoma metastasis to the heart presenting with LV dysfunction and heart failure
j o u r n a l o f i n d i a n c o l l e g e o f c a r d i o l o g y 3 ( 2 0 1 3 ) 6 8 e7 0
Available online at www.sciencedirect.com
journal homepage: www.elsevier.com/locate/jicc
Case Report
Synovial sarcoma metastasis to the heart presenting with LV dysfunction and heart failure N. Rama Kumari a,*, I. Bhaskara Raju b, M. Aruna Devi c, Phani Chakravarty Mutnuru d a
Asso. Professor, Department of Cardiology, Nizam’s Institute of Medical Sciences, Panjagutta, Hyderabad 500 082, Andhra Pradesh, India Professor, Osmania Medical College (Govt. of Andhra Pradesh), Hyderabad 500 095, Andhra Pradesh, India c Assistant Professor in Radiology, Nizam’s Institute of Medical Sciences, Hyderabad 500 082, Andhra Pradesh, India d Senior Resident in Radioliogy, Nizam’s Institute of Medical Sciences, Panjagutta, Hyderabad 500 082, Andhra Pradesh, India b
article info
abstract
Article history:
Soft tissue sarcomas comprise just 1% of all cancers that are diagnosed annually. Synovial
Received 21 March 2013
sarcoma typically arises from Para-articular soft tissues of the extremities and is rarely
Accepted 26 March 2013
found at sites that have no apparent synovial structures. Here we describe a case of sy-
Available online 2 April 2013
novial sarcoma diagnosed pathologically by biopsy from primary site and treated with chemotherapy and radiotherapy. Later, patient presented with metastasis to the heart
Keywords:
causing severe LV dysfunction and heart failure, which was diagnosed by 2D Echo cardi-
Synovial sarcoma
ography, Computed Tomography of chest and Contrast Enhanced Computed Tomography
2D echo
of Abdomen. Patient died and autopsy was done and diagnosis confirmed. Copyright ª 2013, Indian College of Cardiology. All rights reserved.
CT scan Heart failure
1.
Case report
A 35-year-old man was admitted in medical Oncology Department of our hospital with multiple swellings over right thigh and right arm. Biopsy from the swelling showed synovial sarcoma [Fig. 1], which was further confirmed by immunohistochemistry [Fig 2]. He was treated with 6 cycles of chemotherapy. After 3 months he presented with orthopnea (New York Heart Association class 4) and was admitted in Intensive Care Unit (ICU). Cardio Vascular System (CVS) examination was revealed Left Ventricular S3, lungs had bilateral basal crepitations, Electro Cardiogram (ECG) showed sinus tachycardia and poor R wave progression, X-ray showed Cardiomegaly with mild left pleural effusion. 2D Echo four chamber view showed multiple masses attached to the interventricular
septum, Left Ventricular (LV) free wall, Right Ventricular (RV) free wall, Right Atrium and Left Atrium, global hypokinesia of LV and severe LV dysfunction [Fig. 3]. 2D Echo parasternal long axis showed pericardial metastasis [Fig. 4]. Later patient underwent Axial Contrast Enhanced Computed Tomography (CECT) scan of chest and abdomen. CEST chest four-chamber view of heart showed, multiple heterogeneously enhancing deposits involving left atrium, left ventricle, interatrial septum and interventricular septum [Fig. 5]. CECT Abdomen showed patchily enhancing, small, well defined, soft tissue density lesion on right side of pelvis in subcutaneous plane lateral to right greater trochanter involving iliotibial track and gluteus medius muscle s/o metastatic deposits [Fig. 6]. Patient was finally diagnosed to have synovial sarcoma with metastasis to the heart, presenting with heart failure. Patient treated with IV dobutamine, dopamine, digoxin and
* Corresponding author. Tel.: þ91 9866675067, þ91 9440102729; fax: þ91 40 23310076. E-mail addresses: [email protected], [email protected] (N. Rama Kumari). 1561-8811/$ e see front matter Copyright ª 2013, Indian College of Cardiology. All rights reserved. http://dx.doi.org/10.1016/j.jicc.2013.04.002
j o u r n a l o f i n d i a n c o l l e g e o f c a r d i o l o g y 3 ( 2 0 1 3 ) 6 8 e7 0
Fig. 1 e Histo Pathology of synovial sarcoma. Lasix. Patient showed no response, died 5 days after admission. Autopsy was performed, specimen conformed the diagnosis.
69
Fig. 3 e 2D echo e four chamber view showing multiple masses attached to the interventricular septum, LV free wall, RV free wall, RA and LA. Global hypokinesia of LV and severe LV dysfunction.
Synovial sarcoma is prevalent in adolescents and young adults (age range 15e40 years), with a predominance in men.1e3 Primary tumors of the heart and great vessels are rare and usually benign, but approximately 25% are malignant, and most of these are sarcomas. The incidence of primary cardiac sarcomas is 0.0017%e0.19% in unselected autopsy series.4e6 The most common sarcomas are angiosarcoma (31%), rhabdomyosarcoma (21%), malignant mesothelioma (15%), and fibrosarcoma (11%). Metastatic cardiac tumors occur 20e40 times more frequently than primary cardiac neoplasms.4 Microscopically, the classic form of synovial sarcoma is a biphasic tumor that is composed of sharply segregated epithelial and sarcomatous components (in descriptive rather than histogenic terms.). Although a variety of special stains
may aid in tumor identification, definitive diagnosis rests upon the presence of translocation t (X; 18) (p11.2; q11.2), which is exhibited in more than 90% of synovial sarcomas. This translocation can be identified either by means of conventional molecular cytogenetics or by FISH.3 Patients usually present with symptoms of local obstruction or functional interference,7e10 as in our patient. The prognosis is poor and worsens with age. Our patient was already diagnosed as synovial sarcoma and was treated with six cycles of chemotherapy. CECT Chest showed multiple nonhomogenous intra-cardiac masses (involving all chambers and pericardium) are more likely to be metastasis from the synovial sarcoma. Other differential diagnosis are Primary malignant cardiac neoplasms; Angiosarcoma, Rhabdomyosarcoma, Fibrosarcoma, Liposarcoma, Hemangiopericytoma and Fibroushistiocytoma, Primary cardiac lymphoma, Metastasis from lung, breast, Esophageal carcinoma, Malignant melanoma, Malignant germ cell neoplasm and Malignant thymoma. However, they can be differentiated by clinical features and Cardiac MRI. MR imaging, unlike ultrasonography or CT, offers excellent contrast resolution, which allows differentiation between tumor and myocardium. The
Fig. 2 e Immunohistochemistry showing cytokeratin CD 31 and S 100 negative, Bc12 e Positive, correlating morphology with IHC features favor “synovial sarcoma”.
Fig. 4 e 2D echo e parasternal long axis showing metastasis to the pericardium, mild PE, no evidence of pericardial tamponade.
2.
Discussion
70
j o u r n a l o f i n d i a n c o l l e g e o f c a r d i o l o g y 3 ( 2 0 1 3 ) 6 8 e7 0
complete macroscopic resection is possible in only 33% of patients. Recurrence is common even in patients with apparent complete excision.11 After surgical intervention adjuvant radiotherapy for local recurrence and chemotherapy for control of systemic disease might be considered. The most common cause of death is local recurrence (50%) even after complete macroscopic resection. For Multiple metastatic lesions the adjuvant therapy options are radiotherapy and chemotherapy. Cardiac radiation reduces the local recurrence rate and prolongs survival but can lead to long-term cardiac damage.
3. Fig. 5 e Axial CECT chest e showing multiple heterogeneously enhancing soft tissue density lesions with in homogenous enhancement factor with areas of necrosis involving the right atrium, right ventricle, left atrium & left ventricle, interatrial septum and inter ventricle septum.
distinction between thrombus or blood flow artifact can be made more readily with MR imaging than with CT. Signal intensities at MR imaging may also help tissue characterization in some cases. Most cardiac tumors are of low signal intensity on T1-weighted images and are brighter on T2-weighted images. Most malignant disease enhances after administration of contrast material. The distinction between a synovial sarcoma and a primary cardiac mesothelioma of the heart is difficult, and therefore it is important to confirm the t (X; 18) translocation to verify the diagnosis. The currently recommended treatment for localized synovial sarcoma is wide surgical resection to alleviate symptoms and also to avoid potential complications. However,
Fig. 6 e CECT abdomen e showing patchily enhancing, small, well defined, soft tissue density lesion on right side of pelvis in subcutaneous plane lateral to right greater trochanter involving iliotibial track and gluteus medius muscle s/o metastatic deposits.
Conclusions
Echocardiography is an inexpensive and easy screening technique in detecting cardiac tumors. CT and MRI are noninvasive imaging modalities which can delineate cardiac tumors and masses and provide essential information for adequate diagnosis, staging and treatment planning, hence these modalities should be advised wherever available.
Conflicts of interest All authors have none to declare.
references
1. Cihak RA, Lydiatt WM, Lydiatt DD, Bridge JA. Synovial sarcoma of the head and neck: chromosomal translation (X; 18) as a diagnostic aid. Head Neck. 1997 Sep;19(6):549e553. 2. McKinney CD, Mills SE, Fechner RE. Intraarticular synovial sarcoma. Am J Surg Pathol. 1992;16(10):1017e1020. 3. Hazelbag HM, Szuhai K, Tanke HJ, et al. 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(11):1434e1439. 4. Kim CH, Dancer JY, Coffey D, et al. Clinicopathologic study of 24 patients with primary cardiac sarcomas: a 10-year single institution experience. Hum Pathol. 2008;39(6):933e938. 5. Reynen K. Cardiac myxomas. N Engl J Med. 1995;333(24): 1610e1617. 6. Fine G. Neoplasms of the pericardium and heart. In: Gould SE, ed. Pathology of the Heart and Blood Vessels. 3rd ed. Sprinfield (IL): Charles C. Thomas; 1968:851e883. 7. Nazil Y, Karabulut MN, Goya C, et al. Synovial Sarcoma in the right atrium and right ventricle. Acta Cardiol. 2011 Apr;66(2):271e273. 8. Winkler B, Grapow M, Seeberger M, Matt P, Aulitzky W, Eckstein F. Heart failure resulting from gaint left atrial synovial sarcoma metastasis. Thorac Cardiovasc Surg. 2012 Feb;60(1):84e86. 9. Myers P, Konstantinidis S, Karatzas N, Milas F, Panos A. Pericardial synovial sarcoma of the Heart; Is it always worth operating? J Cardiovasc Surg(Torino). 2011 Oct;52(5):749e751. 10. MoorJani N, Peebles C, Gallagher P, Tsang G. Pericardial synovial sarcoma. J Card Surg. 2009 MayeJune;24:349e351. 11. Donsbeck AV, Ranchere D, Coindre JM. Primary cardiac sarcomas; an immunohistochemical and grading study with long-term follow-up of 24 cases. Histopathology. 1999;34(4):295e304.
Available online at www.sciencedirect.com
journal homepage: www.elsevier.com/locate/jicc
Case Report
Synovial sarcoma metastasis to the heart presenting with LV dysfunction and heart failure N. Rama Kumari a,*, I. Bhaskara Raju b, M. Aruna Devi c, Phani Chakravarty Mutnuru d a
Asso. Professor, Department of Cardiology, Nizam’s Institute of Medical Sciences, Panjagutta, Hyderabad 500 082, Andhra Pradesh, India Professor, Osmania Medical College (Govt. of Andhra Pradesh), Hyderabad 500 095, Andhra Pradesh, India c Assistant Professor in Radiology, Nizam’s Institute of Medical Sciences, Hyderabad 500 082, Andhra Pradesh, India d Senior Resident in Radioliogy, Nizam’s Institute of Medical Sciences, Panjagutta, Hyderabad 500 082, Andhra Pradesh, India b
article info
abstract
Article history:
Soft tissue sarcomas comprise just 1% of all cancers that are diagnosed annually. Synovial
Received 21 March 2013
sarcoma typically arises from Para-articular soft tissues of the extremities and is rarely
Accepted 26 March 2013
found at sites that have no apparent synovial structures. Here we describe a case of sy-
Available online 2 April 2013
novial sarcoma diagnosed pathologically by biopsy from primary site and treated with chemotherapy and radiotherapy. Later, patient presented with metastasis to the heart
Keywords:
causing severe LV dysfunction and heart failure, which was diagnosed by 2D Echo cardi-
Synovial sarcoma
ography, Computed Tomography of chest and Contrast Enhanced Computed Tomography
2D echo
of Abdomen. Patient died and autopsy was done and diagnosis confirmed. Copyright ª 2013, Indian College of Cardiology. All rights reserved.
CT scan Heart failure
1.
Case report
A 35-year-old man was admitted in medical Oncology Department of our hospital with multiple swellings over right thigh and right arm. Biopsy from the swelling showed synovial sarcoma [Fig. 1], which was further confirmed by immunohistochemistry [Fig 2]. He was treated with 6 cycles of chemotherapy. After 3 months he presented with orthopnea (New York Heart Association class 4) and was admitted in Intensive Care Unit (ICU). Cardio Vascular System (CVS) examination was revealed Left Ventricular S3, lungs had bilateral basal crepitations, Electro Cardiogram (ECG) showed sinus tachycardia and poor R wave progression, X-ray showed Cardiomegaly with mild left pleural effusion. 2D Echo four chamber view showed multiple masses attached to the interventricular
septum, Left Ventricular (LV) free wall, Right Ventricular (RV) free wall, Right Atrium and Left Atrium, global hypokinesia of LV and severe LV dysfunction [Fig. 3]. 2D Echo parasternal long axis showed pericardial metastasis [Fig. 4]. Later patient underwent Axial Contrast Enhanced Computed Tomography (CECT) scan of chest and abdomen. CEST chest four-chamber view of heart showed, multiple heterogeneously enhancing deposits involving left atrium, left ventricle, interatrial septum and interventricular septum [Fig. 5]. CECT Abdomen showed patchily enhancing, small, well defined, soft tissue density lesion on right side of pelvis in subcutaneous plane lateral to right greater trochanter involving iliotibial track and gluteus medius muscle s/o metastatic deposits [Fig. 6]. Patient was finally diagnosed to have synovial sarcoma with metastasis to the heart, presenting with heart failure. Patient treated with IV dobutamine, dopamine, digoxin and
* Corresponding author. Tel.: þ91 9866675067, þ91 9440102729; fax: þ91 40 23310076. E-mail addresses: [email protected], [email protected] (N. Rama Kumari). 1561-8811/$ e see front matter Copyright ª 2013, Indian College of Cardiology. All rights reserved. http://dx.doi.org/10.1016/j.jicc.2013.04.002
j o u r n a l o f i n d i a n c o l l e g e o f c a r d i o l o g y 3 ( 2 0 1 3 ) 6 8 e7 0
Fig. 1 e Histo Pathology of synovial sarcoma. Lasix. Patient showed no response, died 5 days after admission. Autopsy was performed, specimen conformed the diagnosis.
69
Fig. 3 e 2D echo e four chamber view showing multiple masses attached to the interventricular septum, LV free wall, RV free wall, RA and LA. Global hypokinesia of LV and severe LV dysfunction.
Synovial sarcoma is prevalent in adolescents and young adults (age range 15e40 years), with a predominance in men.1e3 Primary tumors of the heart and great vessels are rare and usually benign, but approximately 25% are malignant, and most of these are sarcomas. The incidence of primary cardiac sarcomas is 0.0017%e0.19% in unselected autopsy series.4e6 The most common sarcomas are angiosarcoma (31%), rhabdomyosarcoma (21%), malignant mesothelioma (15%), and fibrosarcoma (11%). Metastatic cardiac tumors occur 20e40 times more frequently than primary cardiac neoplasms.4 Microscopically, the classic form of synovial sarcoma is a biphasic tumor that is composed of sharply segregated epithelial and sarcomatous components (in descriptive rather than histogenic terms.). Although a variety of special stains
may aid in tumor identification, definitive diagnosis rests upon the presence of translocation t (X; 18) (p11.2; q11.2), which is exhibited in more than 90% of synovial sarcomas. This translocation can be identified either by means of conventional molecular cytogenetics or by FISH.3 Patients usually present with symptoms of local obstruction or functional interference,7e10 as in our patient. The prognosis is poor and worsens with age. Our patient was already diagnosed as synovial sarcoma and was treated with six cycles of chemotherapy. CECT Chest showed multiple nonhomogenous intra-cardiac masses (involving all chambers and pericardium) are more likely to be metastasis from the synovial sarcoma. Other differential diagnosis are Primary malignant cardiac neoplasms; Angiosarcoma, Rhabdomyosarcoma, Fibrosarcoma, Liposarcoma, Hemangiopericytoma and Fibroushistiocytoma, Primary cardiac lymphoma, Metastasis from lung, breast, Esophageal carcinoma, Malignant melanoma, Malignant germ cell neoplasm and Malignant thymoma. However, they can be differentiated by clinical features and Cardiac MRI. MR imaging, unlike ultrasonography or CT, offers excellent contrast resolution, which allows differentiation between tumor and myocardium. The
Fig. 2 e Immunohistochemistry showing cytokeratin CD 31 and S 100 negative, Bc12 e Positive, correlating morphology with IHC features favor “synovial sarcoma”.
Fig. 4 e 2D echo e parasternal long axis showing metastasis to the pericardium, mild PE, no evidence of pericardial tamponade.
2.
Discussion
70
j o u r n a l o f i n d i a n c o l l e g e o f c a r d i o l o g y 3 ( 2 0 1 3 ) 6 8 e7 0
complete macroscopic resection is possible in only 33% of patients. Recurrence is common even in patients with apparent complete excision.11 After surgical intervention adjuvant radiotherapy for local recurrence and chemotherapy for control of systemic disease might be considered. The most common cause of death is local recurrence (50%) even after complete macroscopic resection. For Multiple metastatic lesions the adjuvant therapy options are radiotherapy and chemotherapy. Cardiac radiation reduces the local recurrence rate and prolongs survival but can lead to long-term cardiac damage.
3. Fig. 5 e Axial CECT chest e showing multiple heterogeneously enhancing soft tissue density lesions with in homogenous enhancement factor with areas of necrosis involving the right atrium, right ventricle, left atrium & left ventricle, interatrial septum and inter ventricle septum.
distinction between thrombus or blood flow artifact can be made more readily with MR imaging than with CT. Signal intensities at MR imaging may also help tissue characterization in some cases. Most cardiac tumors are of low signal intensity on T1-weighted images and are brighter on T2-weighted images. Most malignant disease enhances after administration of contrast material. The distinction between a synovial sarcoma and a primary cardiac mesothelioma of the heart is difficult, and therefore it is important to confirm the t (X; 18) translocation to verify the diagnosis. The currently recommended treatment for localized synovial sarcoma is wide surgical resection to alleviate symptoms and also to avoid potential complications. However,
Fig. 6 e CECT abdomen e showing patchily enhancing, small, well defined, soft tissue density lesion on right side of pelvis in subcutaneous plane lateral to right greater trochanter involving iliotibial track and gluteus medius muscle s/o metastatic deposits.
Conclusions
Echocardiography is an inexpensive and easy screening technique in detecting cardiac tumors. CT and MRI are noninvasive imaging modalities which can delineate cardiac tumors and masses and provide essential information for adequate diagnosis, staging and treatment planning, hence these modalities should be advised wherever available.
Conflicts of interest All authors have none to declare.
references
1. Cihak RA, Lydiatt WM, Lydiatt DD, Bridge JA. Synovial sarcoma of the head and neck: chromosomal translation (X; 18) as a diagnostic aid. Head Neck. 1997 Sep;19(6):549e553. 2. McKinney CD, Mills SE, Fechner RE. Intraarticular synovial sarcoma. Am J Surg Pathol. 1992;16(10):1017e1020. 3. Hazelbag HM, Szuhai K, Tanke HJ, et al. 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(11):1434e1439. 4. Kim CH, Dancer JY, Coffey D, et al. Clinicopathologic study of 24 patients with primary cardiac sarcomas: a 10-year single institution experience. Hum Pathol. 2008;39(6):933e938. 5. Reynen K. Cardiac myxomas. N Engl J Med. 1995;333(24): 1610e1617. 6. Fine G. Neoplasms of the pericardium and heart. In: Gould SE, ed. Pathology of the Heart and Blood Vessels. 3rd ed. Sprinfield (IL): Charles C. Thomas; 1968:851e883. 7. Nazil Y, Karabulut MN, Goya C, et al. Synovial Sarcoma in the right atrium and right ventricle. Acta Cardiol. 2011 Apr;66(2):271e273. 8. Winkler B, Grapow M, Seeberger M, Matt P, Aulitzky W, Eckstein F. Heart failure resulting from gaint left atrial synovial sarcoma metastasis. Thorac Cardiovasc Surg. 2012 Feb;60(1):84e86. 9. Myers P, Konstantinidis S, Karatzas N, Milas F, Panos A. Pericardial synovial sarcoma of the Heart; Is it always worth operating? J Cardiovasc Surg(Torino). 2011 Oct;52(5):749e751. 10. MoorJani N, Peebles C, Gallagher P, Tsang G. Pericardial synovial sarcoma. J Card Surg. 2009 MayeJune;24:349e351. 11. Donsbeck AV, Ranchere D, Coindre JM. Primary cardiac sarcomas; an immunohistochemical and grading study with long-term follow-up of 24 cases. Histopathology. 1999;34(4):295e304.