© 2014, Wiley Periodicals, Inc. DOI: 10.1111/echo.12777
Echocardiography
Primary Intimal (Spindle Cell) Sarcoma of the Left Atrium Bo Fu, M.D., Hui Yu, M.D., and Jian Yang, M.D. Department of Cardiovascular Surgery, West China Hospital of Sichuan University, ChengDu, China
(Echocardiography 2015;32:192–194) Key words: mass, sarcoma, color Doppler three-dimensional echocardiography
A previously healthy 50-year-old man presented to our hospital with a history of progressive shortness of breath and diminished exercise tolerance for 3 months. Physical examination was normal. A transthoracic echocardiogram was interpreted as showing a left atrial mass that was mobile and protruding into the left ventricle (LV) and noted that it was “like a myxoma, though its attachment was not visualized.” The preoperative transesophageal echocardiogram (TEE) (Philips iE33, Philips, Andover, MA, USA) revealed a mild enlargement of the left atrium (LA) showing two separate sessile lobular spherical masses occupying almost entirely the LA and arriving just near the posterior mitral annulus (Fig. 1). Of the two masses, one was 2.5 9 3.5 cm and the other was 3.5 9 5.5 cm, the smaller one was partially protruding into the LV in diastole (Fig. 2, movie clip S1), and the possibility of the mass invading the myocardium and epicardium cannot be excluded. Three-dimensional TEE demonstrated the anatomy of tumors (red arrow) and the mitral annulus (Fig. 3). He was taken to the operating room with the initial diagnosis of left atrial myxoma, but upon surgical inspection of the LA, it was noted that the appearance of the tumor was more suggestive of malignancy. Moreover, a small tumor which was not seen in preoperative echocardiography was discovered and sent for frozen diagnosis. The tumors were removed with its stalk and a rim of septum around its base. Macroscopic examination of the cardiac specimen revealed the tumors weighing 28.4 g, and the tumors involved the Hui Yu is co-first author. Address for correspondence and reprint requests: Jian Yang, M.D., Department of Cardiovascular Surgery, West China Hospital of Sichuan University, No. 37, Guoxue Alley, Chengdu 610041, China. Fax: +86-028-85422897; E-mail: [email protected]
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atrial wall posteriorly. The most protuberant part of the tumors was considerably soft in consistency and deeper taupe in color with small darker areas. The cut surface had a nodular, firm, gray–white appearance, small cysts, and areas of hemorrhage (Fig. 4). Biopsy confirmed the surgeon’s suspicion; pathologic examination revealed high-grade densely packed spindle cells replacing and infiltrating into the atrial wall, as well as the epicardial adipose tissue (Fig. 5). The tumor cells showed large, pleomorphic nuclei with prominent nucleoli. An abnormal mitotic figure was identified (Fig. 6). Immunohistochemistry had mild focal positivity for calretinin, MDM2, CDK4, and desmin, and negative staining for myogenin, CD31, CD34, HBME-1, EMA, SMA, and S100; 10% check out for Ki-67. Staining with platelet-derived growth factor was not performed. Primary cardiac tumors are roughly 100 to 1000 times rarer than secondary cardiac neoplasms. About 80% of primary cardiac tumors are benign and 20% are malignant.1 Malignant primaries are most frequently angiosarcomas, rhabdomyosarcomas or fibrosarcomas.2 Malignant tumors are usually asymptomatic until the advanced stages. The most common symptom is congestive heart failure with dyspnea, orthopnea, paroxysmal nocturnal dyspnea, and peripheral edema.3 Intimal (spindle cell) sarcomas are mesenchymal tumors, more commonly encountered in large arterial blood vessels and extremely rare in the heart.4 Although echocardiography is very sensitive in predicting the etiology of most intracavitary cardiac masses, it is less reliable in determining the nature of intramural or extra myocardial lesions. However, three-dimensional echocardiography is very sensitive in determining the presence, site, and nature of a cardiac mass.5,6 The mass and its anatomical relationship with surrounding tissue could be visualized clearly by preoperative
Primary Cardiac Intimal Spindle Cell Sarcoma
Figure 1. Transesophageal echocardiogram demonstrating 2 tumors (yellow arrow) filling the left atrium. A 2.5 9 3.5 cm and 3.5 9 5.5 cm mass seen attached to the posterolateral aspect of the left atrium. LA = left atrium; RA = right atrium; LV = left ventricle; AO = aorta.
Figure 4. Gross pathology of cardiac spindle cell sarcoma.
Figure 5. Section showing sheets of densely packed spindle cells infiltrating into the myocardium (stain: H&E, magnification: 95). Figure 2. Transesophageal echocardiogram demonstrating the smaller tumor (yellow arrow) partially protruding into the left ventricle in diastole. LA = left atrium; LV = left ventricle; LVOT = left ventricular outflow tract.
Figure 6. The tumor cells showing large, pleomorphic nuclei with prominent nucleoli. An abnormal mitotic figure was identified (stain: H&E, magnification: 940).
Figure 3. Three-dimensional transesophageal echocardiogram demonstrating anatomy of tumors (red arrow) and the mitral annulus. LA = left atrium; LV = left ventricle.
three-dimensional image, and operation can be done under three-dimensional TEE guidance.7 The prognosis of cardiac primary intimal (spindle cell) sarcomas is generally poor. The 193
Fu, et al.
effects of a cardiac tumor depend on its anatomical location in the heart, size, invasiveness, friability, and rate of growth. The most important factor affecting the prognosis of these tumors is the anatomic allocation in the heart (intracavitary vs. intra/extra myocardial growth).8 References 1. Perchinsky MJ, Lichtenstein SV, Tyers GF: Primary cardiac tumors: Forty years’ experience with 71 patients. Cancer 1997;79:1809–1815. 2. Ibrahim A, Luk A, Singhal P, et al: Primary intimal (spindle cell) sarcoma of the heart: A case report and review of the literature. Case Rep Med 2013;2013:461815. 3. Shapiro LM: Cardiac tumours: Diagnosis and management. Heart 2001;85:218–222. 4. Kim JB, Kim SH, Lim SY, et al: Primary angiosarcoma of the pulmonary trunk mimicking pulmonary thromboembolism. Echocardiography 2010;27:E23–E26. 5. Khairnar P, Hsiung MC, Mishra S, et al: The ability of live three-dimensional transesophageal echocardiography to
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evaluate the attachment site of intracardiac tumors. Echocardiography 2011;28:1041–1045. 6. Mehmood F, Nanda NC, Vengala S, et al: Live threedimensional transthoracic echocardiographic assessment of left atrial tumors. Echocardiography 2005;22:137–143. 7. Tolstrup K, Shiota T, Gurudevan S, et al: Left atrial myxomas: Correlation of two-dimensional and live three-dimensional transesophageal echocardiography with the clinical and pathologic findings. J Am Soc Echocardiogr 2011;24: 618–624. ~ uela MG, et al: Transtho8. Garg A, Mooney J, Amado Escan racic echocardiographic assessment of spindle cell sarcoma of the pulmonary artery in a child. Echocardiography 2014;31:385–387.
Supporting Information Additional Supporting Information may be found in the online version of this article: Movie clip S1. TEE demonstrating the smaller tumor partially protruding into the left ventricle in diastole.
Copyright of Echocardiography is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
Echocardiography
Primary Intimal (Spindle Cell) Sarcoma of the Left Atrium Bo Fu, M.D., Hui Yu, M.D., and Jian Yang, M.D. Department of Cardiovascular Surgery, West China Hospital of Sichuan University, ChengDu, China
(Echocardiography 2015;32:192–194) Key words: mass, sarcoma, color Doppler three-dimensional echocardiography
A previously healthy 50-year-old man presented to our hospital with a history of progressive shortness of breath and diminished exercise tolerance for 3 months. Physical examination was normal. A transthoracic echocardiogram was interpreted as showing a left atrial mass that was mobile and protruding into the left ventricle (LV) and noted that it was “like a myxoma, though its attachment was not visualized.” The preoperative transesophageal echocardiogram (TEE) (Philips iE33, Philips, Andover, MA, USA) revealed a mild enlargement of the left atrium (LA) showing two separate sessile lobular spherical masses occupying almost entirely the LA and arriving just near the posterior mitral annulus (Fig. 1). Of the two masses, one was 2.5 9 3.5 cm and the other was 3.5 9 5.5 cm, the smaller one was partially protruding into the LV in diastole (Fig. 2, movie clip S1), and the possibility of the mass invading the myocardium and epicardium cannot be excluded. Three-dimensional TEE demonstrated the anatomy of tumors (red arrow) and the mitral annulus (Fig. 3). He was taken to the operating room with the initial diagnosis of left atrial myxoma, but upon surgical inspection of the LA, it was noted that the appearance of the tumor was more suggestive of malignancy. Moreover, a small tumor which was not seen in preoperative echocardiography was discovered and sent for frozen diagnosis. The tumors were removed with its stalk and a rim of septum around its base. Macroscopic examination of the cardiac specimen revealed the tumors weighing 28.4 g, and the tumors involved the Hui Yu is co-first author. Address for correspondence and reprint requests: Jian Yang, M.D., Department of Cardiovascular Surgery, West China Hospital of Sichuan University, No. 37, Guoxue Alley, Chengdu 610041, China. Fax: +86-028-85422897; E-mail: [email protected]
192
atrial wall posteriorly. The most protuberant part of the tumors was considerably soft in consistency and deeper taupe in color with small darker areas. The cut surface had a nodular, firm, gray–white appearance, small cysts, and areas of hemorrhage (Fig. 4). Biopsy confirmed the surgeon’s suspicion; pathologic examination revealed high-grade densely packed spindle cells replacing and infiltrating into the atrial wall, as well as the epicardial adipose tissue (Fig. 5). The tumor cells showed large, pleomorphic nuclei with prominent nucleoli. An abnormal mitotic figure was identified (Fig. 6). Immunohistochemistry had mild focal positivity for calretinin, MDM2, CDK4, and desmin, and negative staining for myogenin, CD31, CD34, HBME-1, EMA, SMA, and S100; 10% check out for Ki-67. Staining with platelet-derived growth factor was not performed. Primary cardiac tumors are roughly 100 to 1000 times rarer than secondary cardiac neoplasms. About 80% of primary cardiac tumors are benign and 20% are malignant.1 Malignant primaries are most frequently angiosarcomas, rhabdomyosarcomas or fibrosarcomas.2 Malignant tumors are usually asymptomatic until the advanced stages. The most common symptom is congestive heart failure with dyspnea, orthopnea, paroxysmal nocturnal dyspnea, and peripheral edema.3 Intimal (spindle cell) sarcomas are mesenchymal tumors, more commonly encountered in large arterial blood vessels and extremely rare in the heart.4 Although echocardiography is very sensitive in predicting the etiology of most intracavitary cardiac masses, it is less reliable in determining the nature of intramural or extra myocardial lesions. However, three-dimensional echocardiography is very sensitive in determining the presence, site, and nature of a cardiac mass.5,6 The mass and its anatomical relationship with surrounding tissue could be visualized clearly by preoperative
Primary Cardiac Intimal Spindle Cell Sarcoma
Figure 1. Transesophageal echocardiogram demonstrating 2 tumors (yellow arrow) filling the left atrium. A 2.5 9 3.5 cm and 3.5 9 5.5 cm mass seen attached to the posterolateral aspect of the left atrium. LA = left atrium; RA = right atrium; LV = left ventricle; AO = aorta.
Figure 4. Gross pathology of cardiac spindle cell sarcoma.
Figure 5. Section showing sheets of densely packed spindle cells infiltrating into the myocardium (stain: H&E, magnification: 95). Figure 2. Transesophageal echocardiogram demonstrating the smaller tumor (yellow arrow) partially protruding into the left ventricle in diastole. LA = left atrium; LV = left ventricle; LVOT = left ventricular outflow tract.
Figure 6. The tumor cells showing large, pleomorphic nuclei with prominent nucleoli. An abnormal mitotic figure was identified (stain: H&E, magnification: 940).
Figure 3. Three-dimensional transesophageal echocardiogram demonstrating anatomy of tumors (red arrow) and the mitral annulus. LA = left atrium; LV = left ventricle.
three-dimensional image, and operation can be done under three-dimensional TEE guidance.7 The prognosis of cardiac primary intimal (spindle cell) sarcomas is generally poor. The 193
Fu, et al.
effects of a cardiac tumor depend on its anatomical location in the heart, size, invasiveness, friability, and rate of growth. The most important factor affecting the prognosis of these tumors is the anatomic allocation in the heart (intracavitary vs. intra/extra myocardial growth).8 References 1. Perchinsky MJ, Lichtenstein SV, Tyers GF: Primary cardiac tumors: Forty years’ experience with 71 patients. Cancer 1997;79:1809–1815. 2. Ibrahim A, Luk A, Singhal P, et al: Primary intimal (spindle cell) sarcoma of the heart: A case report and review of the literature. Case Rep Med 2013;2013:461815. 3. Shapiro LM: Cardiac tumours: Diagnosis and management. Heart 2001;85:218–222. 4. Kim JB, Kim SH, Lim SY, et al: Primary angiosarcoma of the pulmonary trunk mimicking pulmonary thromboembolism. Echocardiography 2010;27:E23–E26. 5. Khairnar P, Hsiung MC, Mishra S, et al: The ability of live three-dimensional transesophageal echocardiography to
194
evaluate the attachment site of intracardiac tumors. Echocardiography 2011;28:1041–1045. 6. Mehmood F, Nanda NC, Vengala S, et al: Live threedimensional transthoracic echocardiographic assessment of left atrial tumors. Echocardiography 2005;22:137–143. 7. Tolstrup K, Shiota T, Gurudevan S, et al: Left atrial myxomas: Correlation of two-dimensional and live three-dimensional transesophageal echocardiography with the clinical and pathologic findings. J Am Soc Echocardiogr 2011;24: 618–624. ~ uela MG, et al: Transtho8. Garg A, Mooney J, Amado Escan racic echocardiographic assessment of spindle cell sarcoma of the pulmonary artery in a child. Echocardiography 2014;31:385–387.
Supporting Information Additional Supporting Information may be found in the online version of this article: Movie clip S1. TEE demonstrating the smaller tumor partially protruding into the left ventricle in diastole.
Copyright of Echocardiography is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
