Ann Thorac Surg 2001;71:712-713
© 2001 The Society of Thoracic Surgeons
Case report
Cardiac pheochromocytoma
Jean-Pierre Meunier, MDa,
Etienne Tatou, MDa,
Alain Bernard, MDa,
Roger Brenot, MDa,
Michel David, MDa
a Service de Chirurgie Cardio-Vasculaire et Service de Chirurgie Digestive et Thoracique, Hôpital du Bocage, Centre Hospitalier-Universitaire de Dijon, Dijon, France
Accepted for publication April 9, 2000.
Address reprint requests to Dr Meunier, Service de Chirurgie Cardio-Vasculaire, Hôpital du Bocage, BP 1542, 2 Bd de Lattre de Tassigny, 21034 Dijon Cedex, France
e-mail: jean-pierre.meunier{at}chu-dijon.fr
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Abstract
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Primary pheochromocytomas of the heart are extremely uncommon. In this report, we present the case of a patient with primary cardiac pheochromocytoma arising from the interatrial septum. Metaiodobenzylguanidine-scintigraphy was negative and diagnosis was confirmed by a positive octreotide scintiscan. The tumor was removed successfully using cardiopulmonary bypass.
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Introduction
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Primary pheochromocytomas of the heart are extremely uncommon. Only five patients with primary cardiac pheochromocytomas arising from the interatrial septum have been reported in the literature [1–5]. In this case report, we describe a patient with a negative metaiodobenzylguanidine-scintigraphy and a positive octreotide scintiscan, that was resected en bloc using cardiopulmonary bypass.
An 18-year-old woman was admitted into the hospital with a 2-month history of headaches and palpitations. Her past medical history was significant for Raynaud’s syndrome, vascular headaches, and ophthalmic migraines. The patient denied any alcohol, tobacco, or narcotic use. Hypertension was confirmed with a high systolic pressure (above 170 mm Hg).
Laboratory values and renal function were within normal limits. Urinary catecholamines and their metabolite were markedly elevated and plasma catecholamines level was normal. The presence of pheochromocytoma was suspected. A Holter was performed revealing supraventricular tachycardia. Abdominal computed tomography and iodine-131 metaiodobenzylguanidine scintigraphy were normal. An indium-111 diethylenetriamine pentaacetic acid octreotide scintiscan (Fig 1) revealed the presence of juxtacardiac somatostatin receptors. Transesophageal echocardiography and magnetic resonance imaging (Fig 2) showed a voluminous cardiac tumor (40 x 30 mm) invading the interatrial septum, and compressing the right atrium and superior vena cava.
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Fig 1. Indium-111 diethylenetriamine pentaacetic acid octreotide scintiscan demonstrating the presence of cardiac somatostatin receptors. (Left side = anterior view; Right side = posterior view.)
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Fig 2. Magnetic resonance imaging showing a voluminous cardiac tumor (40 x 30 mm) arising from the interatrial septum.
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The pericardium was explored in an operation through a median sternotomy confirming the presence of a nodular tumor arising from the interatrial septum. Total cardiopulmonary bypass was established and the patient was cooled to 30°C. A resection en bloc was performed after ligation and section of three vascular pedicles, which originated from the interatrial septum. There was a cleavage level between the right atrium, the left atrium, and the interatrial septum. Therefore, resection was possible without opening any cavity.
Microexamination revealed large polygonal cells with numerous neurosecretory granules. The postoperative follow-up was complicated by an unexplained transitory vascular stroke on the fourth day, and by an acute cholecystitis on day 11, removed by a videolaparoscopic operation.
Blood pressure and the urinary catecholamines level remained normal for 6 months after the operation. Only paroxysmal tachycardia was still present.
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Comment
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Cardiac location is a rare site for extra adrenal pheochromocytoma. Since 1974, 31 cases have been published and only 5 patients [1–5] had a tumor arising from the interatrial septum (as in this case report). Cardiac pheochromocytomas grow either from branchiomeric paraganglia or visceral autonomic paraganglia. Paraganglioma localization is often carried out with the help of metaiodobenzylguanidine scintigraphy [5], which was negative in this case. Only the octreotide scintiscan [6] revealed the presence of abnormal cardiac somatostatin receptors. Therefore, we suggest that the preoperative evaluation in cases of pheochromocytoma should include the octreotide scan. Transesophageal echocardiography and magnetic resonance image scanning are necessary for a successful preoperative analysis of the anatomic extent of the tumor. Some surgeons propose to add cardiac catheterization to visualize the nutrient vessels and the relation of the tumor to the coronary arteries [7].
The high operative mortality of this operation [5, 7] makes rigorous management necessary to prevent serious intraoperative hypertension and arrhythmias. Preoperative preparation should be done with adequate alpha and beta adrenergic blockers, and total cardiopulmonary bypass with cardioplegic arrest should be instituted to isolate the heart from the systemic circulation before manipulation of these vasoactive tumors.
Because of their invasive nature, cardiac pheochromocytomas are often difficult to completely remove and are generally carried out by large resections "en bloc" with disease-free margins needing cardiac reconstruction or transplantation [5, 7].
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References
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Lee H.H., Brenner W.I., Vardhan I., Hyatt J., Terlecki M. Cardiac pheochromocytoma originating in the interatrial septum. Chest 1990;97:760-762.[Abstract/Free Full Text]
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Jeevanandam V., Oz M.C., Shapiro B., Barr M.L., Marboe C., Rose E.A. Surgical management of cardiac pheochromocytoma. Ann Surg 1995;221:415-419.[Medline]
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Lauriero F., Rubini G., D’Addabbo F., Rubini D., Schettini F., D’Addabbo A. I-131 MIBG scintigraphy of neuroectodermal tumors. Comparison between I-131 MIBG and In-111 DTPA-octreotide. Clin Nucl Med 1995;20:243-249.[Medline]
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Abstract
Introduction
