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Multimodality Evaluation of a Rare Intracardiac Tumor: Cardiac Hemangioma
CLINICAL COMMUNICATION TO THE EDITOR
Multimodality Evaluation of a Rare Intracardiac Tumor: Cardiac Hemangioma To the Editor:
CASE REPORT A 25-year-old asymptomatic woman with Turner syndrome, bicuspid aortic valve, and severe pectus excavatum had a transthoracic echocardiogram that was inadequate to assess Funding: None. Conflict of Interest: None. Authorship: All authors had access to the data and a role in writing the manuscript. Requests for reprints should be addressed to Robert J. Siegel, MD, Cardiac Noninvasive Laboratory, Heart Institute, Cedars-Sinai Medical Center, 8700 Beverly Blvd., Room 5623, Los Angeles, CA 90048. E-mail address: [email protected].
her valves or left ventricular function. A cardiac magnetic resonance imaging scan demonstrated a large pedunculated, mobile smooth mass measuring 1.2 cm ⫻ 1.5 cm ⫻ 1.4 cm attached to the distal anteroseptum, with a centrally hypoperfused core and peripheral vascularity after contrast injection (Figure 1, A). Two-dimensional (2D) and threedimensional (3D) transesophageal echocardiography (Phillips Medical Systems, Andover, Mass) demonstrated a mobile, well-circumscribed round mass measuring 1.8 ⫻ 1.6 cm attached to the distal septum by a thin linear stalk with increased Definity echo contrast (Lantheus Medical Imaging, N. Billerica, Mass) uptake in the periphery of the mass consistent with increased vascularity (Figure 1, B, C). 3D images confirmed the findings of 2D transesophageal echocardiography (Figure 1, C). The patient had elective excision of the cardiac mass because of its potential for embolization. Resection of the left ventricular tumor was
Figure 1 (A) Cardiac magnetic resonance imaging: pedunculated, well-circumscribed mobile smooth mass measuring 1.2 cm ⫻ 1.5 cm ⫻ 1.4 cm attached to the distal anteroseptum. (B) Two-dimensional transesophageal echocardiography view at mid-esophageal level demonstrates a circumscribed round mass measuring 1.8 ⫻ 1.6 cm. LV ⫽ left ventricle. (C) Three-dimensional images: the circumscribed mobile round mass with a central hypoperfused core attached to the distal interventricular septum by a linear stalk. (D) Gross image: 1.3 ⫻ 1.3 ⫻ 0.8 cm circumscribed, tan-brow soft mass, arrow identifies the stalk. (E) Photomicrographs: there is proliferation of small-to-medium-sized thin-walled vessels, lined by a single layer of endothelial cells (hematoxylin and eosin [H&E] stain), original magnification ⫻ 100. (F) A higher magnification of the small thin-walled vessels shows cavernous areas and intravascular organizing thrombi, which are diagnostic for mixed cavernous and capillary hemangioma (H&E stain), original magnification ⫻ 600.
0002-9343/$ -see front matter © 2011 Elsevier Inc. All rights reserved.
e4 performed through a right mini thoracotomy incision with Da Vinci robotic (Intuitive Surgical, Inc, Sunnyvale, Calif) assistance utilizing total cardiopulmonary bypass. The histology was diagnostic for a mixed cavernous and capillary hemangioma (Figure 1, E, F).
DISCUSSION Hemangiomas are common benign congenital vascular lesions, but primary cardiac hemangiomas are rare. Five to ten percent of all benign primary cardiac tumors are hemangiomas.1 They most often occur in the skin but are occasionally found in the internal organs. The natural history of these tumors is unpredictable and therefore all resectable lesions should be surgically removed. Histologically, hemangiomas are classified as cavernous, capillary, arteriovenous and mixed variety.1 They can be localized in the atria, the ventricles, the ventricular septum, the pericardium and, very rarely, the mitral valve.1 Although most hemangiomas are asymptomatic, they can present with arrhythmias, pericardial effusion, coronary insufficiency, outflow tract obstruction, embolization, or congestive heart failure.2,3 Sudden cardiac death due to conduction disturbances, as well as cardiac rupture and pericardial tamponade, has been reported.2 The long-term prognosis is favorable after adequate surgical resection. Echocardiography has proven to be the most appropriate screening and diagnostic imaging modality, with an 81% accuracy rate in detecting cardiac tumors.3 3D transesophageal echocardiography added incremental value by clearly identifying the attachment, location, and morphology of the
The American Journal of Medicine, Vol 124, No 8, August 2011 mass more clearly. Cardiac magnetic resonance imaging also is highly accurate in identifying cardiac masses.3 In our case, a cardiac tumor was suspected based on echocardiography and cardiac magnetic resonance imaging, but the nature of the tumor was unknown. Surgical intervention was indicated to identify the type of cardiac mass, establish a tissue diagnosis, and to prevent systemic embolization. Raza H. Orakzai, MDa Smruti Nalawadi, MDa Olivera Cuk, MDa Louise Thomson, MDa Yaron Elad, MDa Alfredo Trento, MDa Robert J. Siegel, MDa Sanam Loghavi, MDb a
b
Heart Institute Department of Pathology and Laboratory Medicine Cedars-Sinai Medical Center Los Angeles, Calif
doi:10.1016/j.amjmed.2011.01.028
References 1. Burke A, Johns JP, Virmani R. Hemangiomas of the heart. A clinicopathologic study of ten cases. Am J Cardiovasc Pathol 1990;3(4): 283-90. 2. Huang CL, Feng AN, Chuang YC, et al. Malignant presentation of cardiac hemangioma: a rare cause of complete atrioventricular block. Circ Cardiovasc Imaging 2008;1(1):e1-3. 3. Esmaeilzadeh M, Jalalian R, Maleki M, Givtaj N, Mozaffari K, Parsaee M. Cardiac cavernous hemangioma. Eur J Echocardiogr 2007;8:487-9.
Multimodality Evaluation of a Rare Intracardiac Tumor: Cardiac Hemangioma To the Editor:
CASE REPORT A 25-year-old asymptomatic woman with Turner syndrome, bicuspid aortic valve, and severe pectus excavatum had a transthoracic echocardiogram that was inadequate to assess Funding: None. Conflict of Interest: None. Authorship: All authors had access to the data and a role in writing the manuscript. Requests for reprints should be addressed to Robert J. Siegel, MD, Cardiac Noninvasive Laboratory, Heart Institute, Cedars-Sinai Medical Center, 8700 Beverly Blvd., Room 5623, Los Angeles, CA 90048. E-mail address: [email protected].
her valves or left ventricular function. A cardiac magnetic resonance imaging scan demonstrated a large pedunculated, mobile smooth mass measuring 1.2 cm ⫻ 1.5 cm ⫻ 1.4 cm attached to the distal anteroseptum, with a centrally hypoperfused core and peripheral vascularity after contrast injection (Figure 1, A). Two-dimensional (2D) and threedimensional (3D) transesophageal echocardiography (Phillips Medical Systems, Andover, Mass) demonstrated a mobile, well-circumscribed round mass measuring 1.8 ⫻ 1.6 cm attached to the distal septum by a thin linear stalk with increased Definity echo contrast (Lantheus Medical Imaging, N. Billerica, Mass) uptake in the periphery of the mass consistent with increased vascularity (Figure 1, B, C). 3D images confirmed the findings of 2D transesophageal echocardiography (Figure 1, C). The patient had elective excision of the cardiac mass because of its potential for embolization. Resection of the left ventricular tumor was
Figure 1 (A) Cardiac magnetic resonance imaging: pedunculated, well-circumscribed mobile smooth mass measuring 1.2 cm ⫻ 1.5 cm ⫻ 1.4 cm attached to the distal anteroseptum. (B) Two-dimensional transesophageal echocardiography view at mid-esophageal level demonstrates a circumscribed round mass measuring 1.8 ⫻ 1.6 cm. LV ⫽ left ventricle. (C) Three-dimensional images: the circumscribed mobile round mass with a central hypoperfused core attached to the distal interventricular septum by a linear stalk. (D) Gross image: 1.3 ⫻ 1.3 ⫻ 0.8 cm circumscribed, tan-brow soft mass, arrow identifies the stalk. (E) Photomicrographs: there is proliferation of small-to-medium-sized thin-walled vessels, lined by a single layer of endothelial cells (hematoxylin and eosin [H&E] stain), original magnification ⫻ 100. (F) A higher magnification of the small thin-walled vessels shows cavernous areas and intravascular organizing thrombi, which are diagnostic for mixed cavernous and capillary hemangioma (H&E stain), original magnification ⫻ 600.
0002-9343/$ -see front matter © 2011 Elsevier Inc. All rights reserved.
e4 performed through a right mini thoracotomy incision with Da Vinci robotic (Intuitive Surgical, Inc, Sunnyvale, Calif) assistance utilizing total cardiopulmonary bypass. The histology was diagnostic for a mixed cavernous and capillary hemangioma (Figure 1, E, F).
DISCUSSION Hemangiomas are common benign congenital vascular lesions, but primary cardiac hemangiomas are rare. Five to ten percent of all benign primary cardiac tumors are hemangiomas.1 They most often occur in the skin but are occasionally found in the internal organs. The natural history of these tumors is unpredictable and therefore all resectable lesions should be surgically removed. Histologically, hemangiomas are classified as cavernous, capillary, arteriovenous and mixed variety.1 They can be localized in the atria, the ventricles, the ventricular septum, the pericardium and, very rarely, the mitral valve.1 Although most hemangiomas are asymptomatic, they can present with arrhythmias, pericardial effusion, coronary insufficiency, outflow tract obstruction, embolization, or congestive heart failure.2,3 Sudden cardiac death due to conduction disturbances, as well as cardiac rupture and pericardial tamponade, has been reported.2 The long-term prognosis is favorable after adequate surgical resection. Echocardiography has proven to be the most appropriate screening and diagnostic imaging modality, with an 81% accuracy rate in detecting cardiac tumors.3 3D transesophageal echocardiography added incremental value by clearly identifying the attachment, location, and morphology of the
The American Journal of Medicine, Vol 124, No 8, August 2011 mass more clearly. Cardiac magnetic resonance imaging also is highly accurate in identifying cardiac masses.3 In our case, a cardiac tumor was suspected based on echocardiography and cardiac magnetic resonance imaging, but the nature of the tumor was unknown. Surgical intervention was indicated to identify the type of cardiac mass, establish a tissue diagnosis, and to prevent systemic embolization. Raza H. Orakzai, MDa Smruti Nalawadi, MDa Olivera Cuk, MDa Louise Thomson, MDa Yaron Elad, MDa Alfredo Trento, MDa Robert J. Siegel, MDa Sanam Loghavi, MDb a
b
Heart Institute Department of Pathology and Laboratory Medicine Cedars-Sinai Medical Center Los Angeles, Calif
doi:10.1016/j.amjmed.2011.01.028
References 1. Burke A, Johns JP, Virmani R. Hemangiomas of the heart. A clinicopathologic study of ten cases. Am J Cardiovasc Pathol 1990;3(4): 283-90. 2. Huang CL, Feng AN, Chuang YC, et al. Malignant presentation of cardiac hemangioma: a rare cause of complete atrioventricular block. Circ Cardiovasc Imaging 2008;1(1):e1-3. 3. Esmaeilzadeh M, Jalalian R, Maleki M, Givtaj N, Mozaffari K, Parsaee M. Cardiac cavernous hemangioma. Eur J Echocardiogr 2007;8:487-9.