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Ann Thorac Surg 1996;61:1845-1847
© 1996 The Society of Thoracic Surgeons

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Case Report

Paraganglioma of the Interatrial Septum

Department of Thoracic and Cardiovascular Surgery, Deborah Heart and Lung Center, Browns Mills, and University of Medicine and Dentistry of New Jersey, Robert Wood Johnson Medical School, New Brunswick, New Jersey

Accepted for publication January 5, 1996.

	Abstract

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The case of a patient undergoing successful resection of an interatrial septal paraganglioma is presented. The diagnosis of an interatrial mass was established preoperatively by echocardiography, ultrafast cine computed tomographic scan, and cardiac catheterization. The tumor was excised in total, and the interatrial septum and the roof of the left atrium were reconstructed using a bovine pericardial patch.

	Introduction

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Primary cardiac paragangliomas are rare tumors occurring in the third to fourth decade [1]. Sex distribution is equal. About 2% of all paragangliomas are located in the chest, and a few are within the pericardium [2]. Surgical experience with this neoplasm is limited. A patient is described with a large interatrial tumor involving the interatrial septum, the roof of the left atrium, and the right upper lobe pulmonary venous–left atrial junction compressing the superior vena caval–right atrial junction.

A 61-year-old woman presented with a 1-year history of dyspnea on exertion and congestive failure. One year before admission, left atrial myxoma was diagnosed by echocardiography. She refused surgical intervention. On physical examination the blood pressure was 150/90 mm Hg and the pulse was 92 beats/min. There was no jugular venous distention or heart murmurs, and the lungs were clear. Chest roentgenogram revealed cardiomegaly without pulmonary edema. Electrocardiogram revealed normal sinus rhythm with first-degree block and atypical left bundle-branch block. Past medical history included hypertension and left jugular vein thrombosis. Transthoracic echocardiography demonstrated a left atrial mass attached to the atrial septum. Ultrafast cine computed tomographic scan showed a 4 x 4-cm atrial mass, related to the septum, with compression of the superior vena cava rightward. Cardiac catheterization revealed a large intracardiac, well-vascularized mass having a tumor blush from a large feeding vessel originating at the left main coronary artery (Fig 1). Coronary arteries were normal.

View larger version (176K): [in this window] [in a new window]   Fig 1. . Left coronary arteriogram in the right anterior oblique projection showing tumor vessel (arrowhead) originating from the left main coronary artery.

View larger version (140K): [in this window] [in a new window]   Fig 2. . Intraoperative transesophageal echocardiogram showing a superiorly located interatrial septal mass compressing the superior vena cava (SVC). (LA = left atrium; RA = right atrium.)

View larger version (154K): [in this window] [in a new window]   Fig 3. . Highly vascular, encapsulated tumor, microscopically composed of moderate-sized round to polygonal cells in small groups with relatively uniform vesicular nuclei and eosinophilic cytoplasm.

	Comment

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Cardiac paraganglionic tumors originate from derivatives of the neural crest in the mediastinum and are similar to tumors of the carotid body, jugular glomus, and organ of Zuckerkandl. Historically, classification of extraadrenal tumors of the paraganglion system was based on the relative hormonal activity of the lesion. Catecholamine-secreting chromaffin tumors (pheochromocytoma) represented 90% of cases and hormonally inactive nonchromaffin tumors (paraganglioma, chemodectoma) 10% of cases.

Current classification systems are based on anatomic distribution, microscopic structure, and innervation [4]. The interrelated ``families'' include brachiomeric, intravagal, aortico-sympathetic, and visceral-autonomic paraganglia. Paragangliomas of the middle medistinum arise from either the branchiomeric (ie, coronary or aortopulmonary) or the visceral-autonomic (ie, atrium or interatrial septum) family.

Blood supply is typically from the left coronary artery [5]. Histologically these tumors have a typical picture of polygonal cells arranged in a characteristic pattern. Ultrastructurally they present granule-storing chief cells. The incidence of malignancy in paragangliomas varies from 12% to 50% depending on the definition of malignancy (ie, local recurrence and distant metastasis versus cellular variation, active mitosis, and local infiltration) [6, 7]. Despite an initial benign clinical and histologic appearance, these tumors may recur and metastasize [8]. Most of these tumors are slow-growing, highly vascular, and locally infiltrative. Their adherence to adjacent structures makes surgical removal difficult.

Based on the highly vascular nature of these tumors, iodine-131–metaiodobenzylguanidine scintigraphy has been used for diagnosis. Dynamic computed tomographic scanning provides adequate anatomic detail for planning the operation. Magnetic resonance imaging can localize cardiac paragangliomas without injection of contrast material or radiopharmaceuticals.

In summary, paragangliomas are rare tumors of the heart with a predilection for the left atrium. Complete surgical excision is the treatment of choice as local recurrence has been documented. Most paragangliomas are benign, with an incidence of malignancy around 10%. As these lesions may be multifocal, whole-body iodine-131–metaiodobenzylguanidine scintigraphy may provide useful information regarding tumor metastasis. This case illustrates successful management of a large interatrial paraganglioma, including complete excision, preservation of the conduction system, and subsequent reconstruction of the atrium.

	References

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1. Marquez JF, James EC, Gardner RJ. The current status of chemodectomas. Am Surg 1977;43:151–8.[Medline]
2. Abad C, Jimenez P, Santana C, et al. Primary cardiac paraganglioma. J Cardiovasc Surg 1992;33:768–72.[Medline]
3. Straus R, Merliss R. Primary tumor of the heart. Arch Pathol Lab Med 1945;39:74–8.
4. Glenner GG, Grimley PM. Tumors of the extra-adrenal paraganglion system (including chemoreceptors). In: HI Firminger, ed. Atlas of tumor pathology. Washington DC: Armed Forces Institute of Pathology, 2nd series, fasc 9, 1974:13–60.
5. Levi B, Cain AS, Dorzab WE. Coronary paraganglioma. Clin Cardiol 1982;5:505–10.[Medline]
6. Weibull JM. On carotid body tumors. Acta Psychiatr Scand 1961;36:497–511.
7. Harrington SW, Clagett OT, Dockerty MB. Tumors of the carotid body: clinical and pathologic considerations of twenty tumors affecting nineteen patients (one bilateral). Ann Surg 1941;114:820–33.[Medline]
8. Cruz PA, Mahidhara S, Ticzon A, Tobon H. Malignant cardiac paraganglioma: follow-up of a case. J Thorac Cardiovasc Surg 1984;87:942–5.[Medline]

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This Article Abstract Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Michael E. Cane Luis D. Berrizbeitia Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Cane, M. E. Articles by McGrath, L. B. Search for Related Content PubMed PubMed Citation Articles by Cane, M. E. Articles by McGrath, L. B.