Gen Thorac Cardiovasc Surg DOI 10.1007/s11748-015-0567-2
CASE REPORT
Diagnostic features of cardiac cavernous hemangioma in the right ventricle on magnetic resonance imaging Satoru Domoto1 • Fumiko Kimura2 • Kazuhiko Uwabe1 • Hiroyuki Koike1 Mimiko Tabata1 • Atsushi Iguchi1 • Hiroshi Niinami1
•
Received: 20 May 2015 / Accepted: 10 June 2015 Ó The Japanese Association for Thoracic Surgery 2015
Abstract Cardiac tumors are rare; however, with recent advances in imaging techniques, they are being diagnosed more frequently with cardiac magnetic resonance (CMR) imaging. We report a case of a cardiac cavernous hemangioma in the right ventricle. This case was diagnosed with CMR imaging based on the characteristic features of peripheral nodular contrast enhancement and progressive centripetal fill-in. CMR imaging also provided useful preoperative anatomical information, showing the relationships among the tumor, tricuspid valve, and right ventricular anterior wall. Keywords Cardiac cavernous hemangioma Cardiac magnetic resonance imaging
Introduction Hemangiomas are common benign congenital vascular lesions of uncertain origin. They most often occur in the skin; however, they are occasionally found in internal organs. Cardiac hemangiomas are extremely rare and account for approximately 5–10 % of benign cardiac tumors [1]. Most affected patients are asymptomatic, and the tumor is discovered incidentally on echocardiography,
& Satoru Domoto [email protected] 1
Department of Cardiovascular Surgery, Saitama International Medical Center, Saitama Medical University, 1397-1 Yamane, Hidaka, Saitama 350-1298, Japan
2
Department of Diagnostic Radiology, Saitama International Medical Center, Saitama Medical University, 1397-1 Yamane, Hidaka, Saitama 350-1298, Japan
computed tomography (CT), cardiac magnetic resonance (CMR) imaging, or at autopsy [2]. With recent advances in imaging techniques, cardiac hemangioma is being diagnosed more frequently with CMR imaging; this is because CMR imaging provides characteristic features of cavernous hemangioma showing marked high intensity on T2weighted and progressive centripetal contrast enhancement [3]. We present a case of cardiac cavernous hemangioma in the right ventricle (RV) diagnosed before surgery and resected using a surgical plan based on CMR imaging findings. A 71-year-old woman was admitted to our department with a diagnosis of a cardiac tumor. Transthoracic echocardiography for a medical checkup had incidentally detected a mass in the RV. The results of general physical examination, electrocardiography, chest radiographs, and laboratory tests were unremarkable. Transthoracic echocardiography revealed a large, fixed, well-defined, encapsulated, homogenous echogenic mass adhered to the anterior wall and apex of the RV. Coronary angiography showed that the main feeding artery of the tumor arose from the right ventricular branch of the right coronary artery. For further characterization of the cardiac mass, CMR imaging was performed on a 1.5-T scanner. Steady-state free precession cine MR imaging demonstrated 55 9 40 9 34-mm sized mass in the RV. It was attached to the anterior RV wall and tricuspid valve with deep involvement of the anterior papillary muscle (Fig. 1). The mass showed marked high intensity on T2-weighted dark blood turbo spin-echo images (Fig. 2a). T1-weighted inversion recovery gradient-echo perfusion imaging following injection of 0.15 mmol/kg of gadoterate meglumine (MagnescopeÒ) showed the findings of peripheral nodular enhancement and progressive centripetal fill-in (Fig. 2b–d). There were no findings of myocardial infiltration, pericardial involvement,
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Gen Thorac Cardiovasc Surg Fig. 1 Four chamber views of steady-state free precession cine magnetic resonance imaging suggesting that the tumor adheres to the anterior cusp of the tricuspid valve (arrow) because they stay in contact both in diastole (a) and systole (b). Short axis views suggesting that the tumor adheres to the right ventricular anterior free wall (arrow) because they stay in contact both in diastole (c) and systole (d). A T2weighted short axis image (f) at the level of middle portion of the left ventricle (a white line on e indicates the scan level) demonstrating that the tumor showed marked high intensity and involved the anterior papillary muscle (arrow). RA right atrium, RV right ventricle
Fig. 2 Short axis views of cardiac magnetic resonance imaging. a T2-weighted dark blood turbo spin-echo image showing that the tumor (arrow) has a homogeneous high-signal intensity over the entire mass. T1-weighted inversion recovery gradient-echo perfusion imaging following injection of 0.15 mmol/kg of gadoterate
123
meglumine demonstrating the findings of peripheral nodular enhancement and progressive centripetal fill-in. b 30 s after the gadolinium administration, c 60 s after the gadolinium administration. d Hemangioma filled in late phase of gadolinium first-pass perfusion due to delayed venous filling. RV right ventricle, LV left ventricle
Gen Thorac Cardiovasc Surg
or extracardiac extension. Based on the CMR imaging findings, we diagnosed it as a benign cavernous hemangioma with adhesion to the anterior wall of the RV, anterior cusp, and the papillary muscle of the tricuspid valve. Surgery was scheduled for tumor resection and pathological diagnosis. Through a median sternotomy, a cardiopulmonary bypass was established and cardioplegic arrest was achieved. We examined the right ventricle through the tricuspid valve after right atriotomy. The tumor was elastic, originated from the RV free wall, and extended to the apex broadly. As demonstrated on CMR imaging, the tumor adhered to the anterior cusp of the tricuspid valve and involved the anterior papillary muscle. We performed an intra-operative rapid pathologic diagnosis and confirmed the tumor as a benign cavernous hemangioma. We cut the anterior cusp of the tricuspid valve partially and resected the tumor as much as possible. Since the tumor was benign, a total resection and reconstruction of the RV was considered to be an overly invasive surgery. After the partial resection, we applied cryoablation to the resected marginal side of the RV to destroy any remaining tumor tissue on the RV, and repaired the tricuspid valve. Histopathological examination confirmed the tumor as a benign cavernous hemangioma (Fig. 3). The postoperative course was uneventful.
Discussion Cardiac hemangiomas are composed of a benign proliferation of endothelial cells of the blood vessels. They are histologically identical to hemangiomas that arise elsewhere in the body [4]. Cardiac hemangiomas are histologically classified into three types: tumor composed of multiple, dilated, thin-walled vessels (cavernous type); smaller capillary-like vessels (capillary type); and dysplastic malformed arteries and veins (arteriovenous type). Most cardiac hemangiomas are asymptomatic and are discovered incidentally on echocardiography, CT, CMR, or at autopsy. The symptoms depend on the anatomic location and size of the tumor. In symptomatic patients, cardiac hemangiomas cause arrhythmias, pericardial effusions, congestive heart failure, right ventricular outflow tract obstruction, coronary insufficiency, or sudden death. The excellent contrast resolution and multiplanar capability of CMR imaging allows qualitative diagnosis and optimal anatomical evaluation of any cardiac mass. Features such as myocardial infiltration, relation to the cardiac valve, pericardial involvement and extracardiac extension can be evaluated with CMR imaging. In our case, CMR imaging yielded the characteristic findings of hemangioma, which were marked high intensity on T2-weighted images and
Fig. 3 a Photograph showing the tumor (blue arrow) adhered to the anterior cusp of the tricuspid valve and involving the anterior papillary muscle (red arrow). b The tan, bosselated mass was excised at surgery. c Histopathological features were consistent with a cavernous type of hemangioma composed of large, endotheliallined, thin-wall channels (H&E, 920). d Immunohistochemical staining for CD31 confirming the cavernous type of hemangioma. TV tricuspid valve
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Gen Thorac Cardiovasc Surg
gradual centripetal contrast enhancement. These images allowed an accurate pre-surgical diagnosis of cardiac hemangioma. In addition, CMR imaging enabled us to demonstrate the precise relationship among the tumor, tricuspid valve, and RV anterior free wall; this anatomical information was useful for pre-surgical planning. The outcomes of cardiac hemangioma are unpredictable. Patients with resectable tumor usually have a good prognosis; however, the prognosis of patients with unresectable tumors is controversial. It has been reported that patients with an unresectable tumor may have a poor prognosis because of ventricular tachycardia, sudden death, local progression, or systemic dissemination of the tumor [5]. On the other hand, Brizard et al. suggested that long-term prognosis after resection is favorable. When extensive resection requires complex hazard surgical procedures, it should only be performed in severely symptomatic patients to eliminate compression of vital cardiac structure [6]. In the present case, we performed a partial resection because the patient was asymptomatic, and a total resection and reconstruction of the RV was thought to be an overly might invasive surgery.
Conclusion Although cardiac hemangiomas are rare, CMR imaging allows for an accurate diagnosis based on characteristic findings and enables demonstration of the precise
123
anatomical relationships among the tumor, tricuspid valve, and RV free wall. Conflict of interest
We have no conflict of interest.
References 1. Grebenc ML, Rosado de Christenson ML, Burke AP, Green CE, Galvin FR. Primary cardiac and pericardial neoplasms : radiologic–pathologic correlation. Radiographics. 2000;20:1073–103. 2. Burke A, Johns JP, Virimani R. Hemangiomas of the heart. A clinicopathologic study of ten cases. Am J Cardiovasc Pathol. 1990;3:283–90. 3. Tomasian A, Iv M, Lai C, Jalili M, Krishnam MS. Cardiac hemangioma: features of cardiovascular magnetic resonance. J Cardiovasc Magn Reson. 2007;9:873–6. 4. McAllister HA, Fenoglio JJ Jr. Tumors of the cardiovascular system. In: Atlas of tumor pathology, 2nd series, fascicle 15. Washington, DC: Armed Forces Institute of Pathology; 1978. p. 46–52. 5. Tomizawa Y, Endo M, Nishida H, Kikuchi C, Koyanagi H. Reconstruction of the left ventricle in a patient with cardiac hemangioma at the apex. Ann Thorac Surg. 2001;71:2032–4. 6. Brizard C, Latremouille C, Jebara VA, Acar C, Fabiani J, Deloche A, Carpentier AF. Cardiac hemangiomas. Ann Thorac Surg. 1993;56:390–4.
CASE REPORT
Diagnostic features of cardiac cavernous hemangioma in the right ventricle on magnetic resonance imaging Satoru Domoto1 • Fumiko Kimura2 • Kazuhiko Uwabe1 • Hiroyuki Koike1 Mimiko Tabata1 • Atsushi Iguchi1 • Hiroshi Niinami1
•
Received: 20 May 2015 / Accepted: 10 June 2015 Ó The Japanese Association for Thoracic Surgery 2015
Abstract Cardiac tumors are rare; however, with recent advances in imaging techniques, they are being diagnosed more frequently with cardiac magnetic resonance (CMR) imaging. We report a case of a cardiac cavernous hemangioma in the right ventricle. This case was diagnosed with CMR imaging based on the characteristic features of peripheral nodular contrast enhancement and progressive centripetal fill-in. CMR imaging also provided useful preoperative anatomical information, showing the relationships among the tumor, tricuspid valve, and right ventricular anterior wall. Keywords Cardiac cavernous hemangioma Cardiac magnetic resonance imaging
Introduction Hemangiomas are common benign congenital vascular lesions of uncertain origin. They most often occur in the skin; however, they are occasionally found in internal organs. Cardiac hemangiomas are extremely rare and account for approximately 5–10 % of benign cardiac tumors [1]. Most affected patients are asymptomatic, and the tumor is discovered incidentally on echocardiography,
& Satoru Domoto [email protected] 1
Department of Cardiovascular Surgery, Saitama International Medical Center, Saitama Medical University, 1397-1 Yamane, Hidaka, Saitama 350-1298, Japan
2
Department of Diagnostic Radiology, Saitama International Medical Center, Saitama Medical University, 1397-1 Yamane, Hidaka, Saitama 350-1298, Japan
computed tomography (CT), cardiac magnetic resonance (CMR) imaging, or at autopsy [2]. With recent advances in imaging techniques, cardiac hemangioma is being diagnosed more frequently with CMR imaging; this is because CMR imaging provides characteristic features of cavernous hemangioma showing marked high intensity on T2weighted and progressive centripetal contrast enhancement [3]. We present a case of cardiac cavernous hemangioma in the right ventricle (RV) diagnosed before surgery and resected using a surgical plan based on CMR imaging findings. A 71-year-old woman was admitted to our department with a diagnosis of a cardiac tumor. Transthoracic echocardiography for a medical checkup had incidentally detected a mass in the RV. The results of general physical examination, electrocardiography, chest radiographs, and laboratory tests were unremarkable. Transthoracic echocardiography revealed a large, fixed, well-defined, encapsulated, homogenous echogenic mass adhered to the anterior wall and apex of the RV. Coronary angiography showed that the main feeding artery of the tumor arose from the right ventricular branch of the right coronary artery. For further characterization of the cardiac mass, CMR imaging was performed on a 1.5-T scanner. Steady-state free precession cine MR imaging demonstrated 55 9 40 9 34-mm sized mass in the RV. It was attached to the anterior RV wall and tricuspid valve with deep involvement of the anterior papillary muscle (Fig. 1). The mass showed marked high intensity on T2-weighted dark blood turbo spin-echo images (Fig. 2a). T1-weighted inversion recovery gradient-echo perfusion imaging following injection of 0.15 mmol/kg of gadoterate meglumine (MagnescopeÒ) showed the findings of peripheral nodular enhancement and progressive centripetal fill-in (Fig. 2b–d). There were no findings of myocardial infiltration, pericardial involvement,
123
Gen Thorac Cardiovasc Surg Fig. 1 Four chamber views of steady-state free precession cine magnetic resonance imaging suggesting that the tumor adheres to the anterior cusp of the tricuspid valve (arrow) because they stay in contact both in diastole (a) and systole (b). Short axis views suggesting that the tumor adheres to the right ventricular anterior free wall (arrow) because they stay in contact both in diastole (c) and systole (d). A T2weighted short axis image (f) at the level of middle portion of the left ventricle (a white line on e indicates the scan level) demonstrating that the tumor showed marked high intensity and involved the anterior papillary muscle (arrow). RA right atrium, RV right ventricle
Fig. 2 Short axis views of cardiac magnetic resonance imaging. a T2-weighted dark blood turbo spin-echo image showing that the tumor (arrow) has a homogeneous high-signal intensity over the entire mass. T1-weighted inversion recovery gradient-echo perfusion imaging following injection of 0.15 mmol/kg of gadoterate
123
meglumine demonstrating the findings of peripheral nodular enhancement and progressive centripetal fill-in. b 30 s after the gadolinium administration, c 60 s after the gadolinium administration. d Hemangioma filled in late phase of gadolinium first-pass perfusion due to delayed venous filling. RV right ventricle, LV left ventricle
Gen Thorac Cardiovasc Surg
or extracardiac extension. Based on the CMR imaging findings, we diagnosed it as a benign cavernous hemangioma with adhesion to the anterior wall of the RV, anterior cusp, and the papillary muscle of the tricuspid valve. Surgery was scheduled for tumor resection and pathological diagnosis. Through a median sternotomy, a cardiopulmonary bypass was established and cardioplegic arrest was achieved. We examined the right ventricle through the tricuspid valve after right atriotomy. The tumor was elastic, originated from the RV free wall, and extended to the apex broadly. As demonstrated on CMR imaging, the tumor adhered to the anterior cusp of the tricuspid valve and involved the anterior papillary muscle. We performed an intra-operative rapid pathologic diagnosis and confirmed the tumor as a benign cavernous hemangioma. We cut the anterior cusp of the tricuspid valve partially and resected the tumor as much as possible. Since the tumor was benign, a total resection and reconstruction of the RV was considered to be an overly invasive surgery. After the partial resection, we applied cryoablation to the resected marginal side of the RV to destroy any remaining tumor tissue on the RV, and repaired the tricuspid valve. Histopathological examination confirmed the tumor as a benign cavernous hemangioma (Fig. 3). The postoperative course was uneventful.
Discussion Cardiac hemangiomas are composed of a benign proliferation of endothelial cells of the blood vessels. They are histologically identical to hemangiomas that arise elsewhere in the body [4]. Cardiac hemangiomas are histologically classified into three types: tumor composed of multiple, dilated, thin-walled vessels (cavernous type); smaller capillary-like vessels (capillary type); and dysplastic malformed arteries and veins (arteriovenous type). Most cardiac hemangiomas are asymptomatic and are discovered incidentally on echocardiography, CT, CMR, or at autopsy. The symptoms depend on the anatomic location and size of the tumor. In symptomatic patients, cardiac hemangiomas cause arrhythmias, pericardial effusions, congestive heart failure, right ventricular outflow tract obstruction, coronary insufficiency, or sudden death. The excellent contrast resolution and multiplanar capability of CMR imaging allows qualitative diagnosis and optimal anatomical evaluation of any cardiac mass. Features such as myocardial infiltration, relation to the cardiac valve, pericardial involvement and extracardiac extension can be evaluated with CMR imaging. In our case, CMR imaging yielded the characteristic findings of hemangioma, which were marked high intensity on T2-weighted images and
Fig. 3 a Photograph showing the tumor (blue arrow) adhered to the anterior cusp of the tricuspid valve and involving the anterior papillary muscle (red arrow). b The tan, bosselated mass was excised at surgery. c Histopathological features were consistent with a cavernous type of hemangioma composed of large, endotheliallined, thin-wall channels (H&E, 920). d Immunohistochemical staining for CD31 confirming the cavernous type of hemangioma. TV tricuspid valve
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Gen Thorac Cardiovasc Surg
gradual centripetal contrast enhancement. These images allowed an accurate pre-surgical diagnosis of cardiac hemangioma. In addition, CMR imaging enabled us to demonstrate the precise relationship among the tumor, tricuspid valve, and RV anterior free wall; this anatomical information was useful for pre-surgical planning. The outcomes of cardiac hemangioma are unpredictable. Patients with resectable tumor usually have a good prognosis; however, the prognosis of patients with unresectable tumors is controversial. It has been reported that patients with an unresectable tumor may have a poor prognosis because of ventricular tachycardia, sudden death, local progression, or systemic dissemination of the tumor [5]. On the other hand, Brizard et al. suggested that long-term prognosis after resection is favorable. When extensive resection requires complex hazard surgical procedures, it should only be performed in severely symptomatic patients to eliminate compression of vital cardiac structure [6]. In the present case, we performed a partial resection because the patient was asymptomatic, and a total resection and reconstruction of the RV was thought to be an overly might invasive surgery.
Conclusion Although cardiac hemangiomas are rare, CMR imaging allows for an accurate diagnosis based on characteristic findings and enables demonstration of the precise
123
anatomical relationships among the tumor, tricuspid valve, and RV free wall. Conflict of interest
We have no conflict of interest.
References 1. Grebenc ML, Rosado de Christenson ML, Burke AP, Green CE, Galvin FR. Primary cardiac and pericardial neoplasms : radiologic–pathologic correlation. Radiographics. 2000;20:1073–103. 2. Burke A, Johns JP, Virimani R. Hemangiomas of the heart. A clinicopathologic study of ten cases. Am J Cardiovasc Pathol. 1990;3:283–90. 3. Tomasian A, Iv M, Lai C, Jalili M, Krishnam MS. Cardiac hemangioma: features of cardiovascular magnetic resonance. J Cardiovasc Magn Reson. 2007;9:873–6. 4. McAllister HA, Fenoglio JJ Jr. Tumors of the cardiovascular system. In: Atlas of tumor pathology, 2nd series, fascicle 15. Washington, DC: Armed Forces Institute of Pathology; 1978. p. 46–52. 5. Tomizawa Y, Endo M, Nishida H, Kikuchi C, Koyanagi H. Reconstruction of the left ventricle in a patient with cardiac hemangioma at the apex. Ann Thorac Surg. 2001;71:2032–4. 6. Brizard C, Latremouille C, Jebara VA, Acar C, Fabiani J, Deloche A, Carpentier AF. Cardiac hemangiomas. Ann Thorac Surg. 1993;56:390–4.
