- Email: [email protected]
An Incidentaloma in the Cardiology Clinic
CLINICAL COMMUNICATION TO THE EDITOR
An Incidentaloma in the Cardiology Clinic To the Editor: Paragangliomas are rare neuroendocrine tumors of neural crest origin.1,2 In this report, we describe the exhaustive diagnostic workup involved in the diagnosis of a paraganglioma, which was first noted as an incidental mass in the proximity of left atrium on a routine transthoracic echocardiogram (TTE).
CASE REPORT A 55-year-old man with a history of valvular cardiomyopathy, hypertension, diabetes mellitus, and nonobstructive coronary artery disease presented to the cardiology clinic for follow-up after undergoing bioprosthetic aortic valve replacement for severe aortic regurgitation 2 years ago. He denied any dyspnea, palpitations, chest pain, or syncope. Physical examination was remarkable for blood pressure of 127/85 mm Hg and an early-peaking systolic murmur at the right upper sternal border. He was taking aspirin, simvastatin, carvedilol, losartan, and furosemide. A baseline TTE was ordered for follow-up on the bioprosthetic valve function. On the TTE, the left ventricular ejection fraction and the bioprosthetic valve function were normal, but an incidental well-circumscribed mass measuring 35 22 mm was noted in the proximity of the left atrium (Figure, A). On the basis of the TTE images, it could not be ascertained whether the mass was intracardiac or extracardiac. Therefore, a transesophageal echocardiogram was performed, which revealed similar characteristics of the mass as on the TTE, but it was also limited in localizing the lesion (Figure, B, Video, A, available online). Color Doppler (Video, B, available online) suggested a highly vascular mass, concerning for malignancy. Thus, the patient was sent for a cardiac magnetic resonance imaging for further evaluation, which demonstrated a 37 24 47-mm extracardiac oval mass adjacent to the posterior wall of the left atrium with
Funding: None. Conflict of Interest: None. Authorship: All authors had access to the data and played a role in writing this manuscript. Requests for reprints should be addressed to Rupak Desai, MBBS, Atlanta Veterans Affairs Medical Center, 1670 Clairmont Rd, Decatur, GA 30033. E-mail address: [email protected] 0002-9343/$ -see front matter Ó 2016 Elsevier Inc. All rights reserved.
homogenously increased T2 signal intensity (Figure, C). On postcontrast T1 fat-saturated image, the mass had significant homogeneous enhancement (Figure, D). The differential diagnosis for this mass included lymphangioma, teratoma, hemangioma, or paraganglioma, or, less likely, lymphoma, pericardial mesothelioma, or metastatic tumor. The patient did not have any systemic signs or symptoms to suggest a malignant process. To further characterize the mass, a positron emission tomography computed tomography scan was completed. The mass was noted to have a maximum standardized uptake value of 18.7, suggestive of a malignant process (Figure, E). Video-assisted thoracic surgery was performed to biopsy the mass. The gross architecture of the obtained specimen showed a neoplasm forming nests (Figure, F). The cells were mildly pleomorphic with smudgy nuclei. Otherwise, the nuclei had a relatively homogenous chromatin pattern with barely visible nucleoli. The specimen was stained with antibodies directed against cytokeratin, chromogranin, synaptophysin, OCT-4, C-KIT, CD20, and S-100. The neoplastic cells were positive only for chromogranin (Figure, G) and synaptophysin (Figure, H), suggesting a diagnosis of paraganglioma. Elevated urine and plasma metanephrines with normal parathyroid hormone and calcitonin levels were consistent with functioning pheochromocytoma. A 123I-metaiodobenzylguanidine scan was completed, which revealed localized disease as described before without metastasis.
DISCUSSION Intracardiac or extracardiac paraganglioma is a rare tumor of neural crest origin.1,2 The presentation varies on the basis of the functional status and location of the tumor. Most of the mediastinal paragangliomas are nonfunctional, and approximately 90% of them are benign.3 The usual presentation is due to the mass-like effect of the tumor on the surrounding structures causing dyspnea, dysphagia, or ischemic symptoms. Surgical resection of functioning paraganglioma can be complicated because of the location and vascular nature of the tumor.
CONCLUSIONS Because of the location of the tumor and the patient’s history of a sternotomy, surgical resection was technically difficult. The patient was given alpha-blockers and referred for 131 I-metaiodobenzylguanidine treatment. This case underscores
e150
The American Journal of Medicine, Vol 130, No 4, April 2017
Figure A, The mass in the proximity of the left atrium in the 4-chamber view on TTE (white arrow). B, The mass in the proximity of the left atrium on TEE (white arrow). C, Four-chamber view. T2-weighted magnetic resonance imaging demonstrates homogeneous increased T2 signal intensity of the mass (white arrow). D, Four-chamber postcontrast T1 fat-saturated image demonstrates significant homogeneous enhancement of the mass (white arrow). E, Positron emission tomography image showing significant diffuse homogeneous 18F-fluoro-2-deoxy-D-glucose uptake throughout the mass. F, 10 hematoxylineeosin stain of the tumor; tumor cells are in a nested growth pattern and separated by pink sclerotic collagenous stroma with well-vascularized lesion. G, 10 chromogranin immunohistochemical stain. H, 10 synaptophysin immunohistochemical stain.
the challenges associated with diagnosis and management of intracardiac or extracardiac paragangliomas. a
Danesh K. Kella, MD Rupak Desai, MBBSb Leon Rubinsztein, MDc Jeranfel Hernandez, MDc Andro Kacharava, MD, PhDa a
Department of Medicine Emory University School of Medicine Atlanta, Ga b Department of Cardiology Atlanta Veterans Affairs Medical Center Ga c Department of Radiology Atlanta Veterans Affairs Medical Center Ga
http://dx.doi.org/10.1016/j.amjmed.2016.10.033
References 1. Mandak JS, Benoit CH, Starkey RH, Nassef LA. Echocardiography in the evaluation of cardiac pheochromocytoma. Am Heart J. 1996;132: 1063-1066. 2. Wychulis AR, Payne WS, Clagett OT, Woolner LB. Surgical treatment of mediastinal tumors: a 40 year experience. J Thorac Cardiovasc Surg. 1971;62:379-392. 3. Khan MF, Datta S, Chisti MM, Movahed MR. Cardiac paraganglioma: clinical presentation, diagnostic approach and factors affecting short and long-term outcomes. Int J Cardiol. 2013;166:315-320.
SUPPLEMENTARY DATA Supplementary Videos accompanying this article can be found in the online version at http://dx.doi.org/10.1016/j. amjmed.2016.10.033.
An Incidentaloma in the Cardiology Clinic To the Editor: Paragangliomas are rare neuroendocrine tumors of neural crest origin.1,2 In this report, we describe the exhaustive diagnostic workup involved in the diagnosis of a paraganglioma, which was first noted as an incidental mass in the proximity of left atrium on a routine transthoracic echocardiogram (TTE).
CASE REPORT A 55-year-old man with a history of valvular cardiomyopathy, hypertension, diabetes mellitus, and nonobstructive coronary artery disease presented to the cardiology clinic for follow-up after undergoing bioprosthetic aortic valve replacement for severe aortic regurgitation 2 years ago. He denied any dyspnea, palpitations, chest pain, or syncope. Physical examination was remarkable for blood pressure of 127/85 mm Hg and an early-peaking systolic murmur at the right upper sternal border. He was taking aspirin, simvastatin, carvedilol, losartan, and furosemide. A baseline TTE was ordered for follow-up on the bioprosthetic valve function. On the TTE, the left ventricular ejection fraction and the bioprosthetic valve function were normal, but an incidental well-circumscribed mass measuring 35 22 mm was noted in the proximity of the left atrium (Figure, A). On the basis of the TTE images, it could not be ascertained whether the mass was intracardiac or extracardiac. Therefore, a transesophageal echocardiogram was performed, which revealed similar characteristics of the mass as on the TTE, but it was also limited in localizing the lesion (Figure, B, Video, A, available online). Color Doppler (Video, B, available online) suggested a highly vascular mass, concerning for malignancy. Thus, the patient was sent for a cardiac magnetic resonance imaging for further evaluation, which demonstrated a 37 24 47-mm extracardiac oval mass adjacent to the posterior wall of the left atrium with
Funding: None. Conflict of Interest: None. Authorship: All authors had access to the data and played a role in writing this manuscript. Requests for reprints should be addressed to Rupak Desai, MBBS, Atlanta Veterans Affairs Medical Center, 1670 Clairmont Rd, Decatur, GA 30033. E-mail address: [email protected] 0002-9343/$ -see front matter Ó 2016 Elsevier Inc. All rights reserved.
homogenously increased T2 signal intensity (Figure, C). On postcontrast T1 fat-saturated image, the mass had significant homogeneous enhancement (Figure, D). The differential diagnosis for this mass included lymphangioma, teratoma, hemangioma, or paraganglioma, or, less likely, lymphoma, pericardial mesothelioma, or metastatic tumor. The patient did not have any systemic signs or symptoms to suggest a malignant process. To further characterize the mass, a positron emission tomography computed tomography scan was completed. The mass was noted to have a maximum standardized uptake value of 18.7, suggestive of a malignant process (Figure, E). Video-assisted thoracic surgery was performed to biopsy the mass. The gross architecture of the obtained specimen showed a neoplasm forming nests (Figure, F). The cells were mildly pleomorphic with smudgy nuclei. Otherwise, the nuclei had a relatively homogenous chromatin pattern with barely visible nucleoli. The specimen was stained with antibodies directed against cytokeratin, chromogranin, synaptophysin, OCT-4, C-KIT, CD20, and S-100. The neoplastic cells were positive only for chromogranin (Figure, G) and synaptophysin (Figure, H), suggesting a diagnosis of paraganglioma. Elevated urine and plasma metanephrines with normal parathyroid hormone and calcitonin levels were consistent with functioning pheochromocytoma. A 123I-metaiodobenzylguanidine scan was completed, which revealed localized disease as described before without metastasis.
DISCUSSION Intracardiac or extracardiac paraganglioma is a rare tumor of neural crest origin.1,2 The presentation varies on the basis of the functional status and location of the tumor. Most of the mediastinal paragangliomas are nonfunctional, and approximately 90% of them are benign.3 The usual presentation is due to the mass-like effect of the tumor on the surrounding structures causing dyspnea, dysphagia, or ischemic symptoms. Surgical resection of functioning paraganglioma can be complicated because of the location and vascular nature of the tumor.
CONCLUSIONS Because of the location of the tumor and the patient’s history of a sternotomy, surgical resection was technically difficult. The patient was given alpha-blockers and referred for 131 I-metaiodobenzylguanidine treatment. This case underscores
e150
The American Journal of Medicine, Vol 130, No 4, April 2017
Figure A, The mass in the proximity of the left atrium in the 4-chamber view on TTE (white arrow). B, The mass in the proximity of the left atrium on TEE (white arrow). C, Four-chamber view. T2-weighted magnetic resonance imaging demonstrates homogeneous increased T2 signal intensity of the mass (white arrow). D, Four-chamber postcontrast T1 fat-saturated image demonstrates significant homogeneous enhancement of the mass (white arrow). E, Positron emission tomography image showing significant diffuse homogeneous 18F-fluoro-2-deoxy-D-glucose uptake throughout the mass. F, 10 hematoxylineeosin stain of the tumor; tumor cells are in a nested growth pattern and separated by pink sclerotic collagenous stroma with well-vascularized lesion. G, 10 chromogranin immunohistochemical stain. H, 10 synaptophysin immunohistochemical stain.
the challenges associated with diagnosis and management of intracardiac or extracardiac paragangliomas. a
Danesh K. Kella, MD Rupak Desai, MBBSb Leon Rubinsztein, MDc Jeranfel Hernandez, MDc Andro Kacharava, MD, PhDa a
Department of Medicine Emory University School of Medicine Atlanta, Ga b Department of Cardiology Atlanta Veterans Affairs Medical Center Ga c Department of Radiology Atlanta Veterans Affairs Medical Center Ga
http://dx.doi.org/10.1016/j.amjmed.2016.10.033
References 1. Mandak JS, Benoit CH, Starkey RH, Nassef LA. Echocardiography in the evaluation of cardiac pheochromocytoma. Am Heart J. 1996;132: 1063-1066. 2. Wychulis AR, Payne WS, Clagett OT, Woolner LB. Surgical treatment of mediastinal tumors: a 40 year experience. J Thorac Cardiovasc Surg. 1971;62:379-392. 3. Khan MF, Datta S, Chisti MM, Movahed MR. Cardiac paraganglioma: clinical presentation, diagnostic approach and factors affecting short and long-term outcomes. Int J Cardiol. 2013;166:315-320.
SUPPLEMENTARY DATA Supplementary Videos accompanying this article can be found in the online version at http://dx.doi.org/10.1016/j. amjmed.2016.10.033.