Ann Thorac Surg 1997;64:1824-1826
© 1997 The Society of Thoracic Surgeons
Case Report
Resection of Multiple Mediastinal Paragangliomas With Cardiopulmonary Bypass
John A. Kern, MD,
Todd A. Milbrandt, MD,
Sheri Rolf, MD,
Curtis G. Tribble, MD
Department of Thoracic and Cardiovascular Surgery, University of Virginia Health Sciences Center, Charlottesville, Virginia
Accepted for publication July 23, 1997.
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Abstract
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The case of a patient who underwent simultaneous resection of two separate mediastinal paragangliomas is presented. The tumors were located in the aortopulmonary window and in the right atrioventricular groove. The patient had previously undergone resection of a glomus vagale tumor. The mediastinal lesions were removed with the aid of cardiopulmonary bypass, and the patient is doing well postoperatively.
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Introduction
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Paragangliomas are tumors that arise from cells associated with the autonomic nervous system. These tumors are rare and are usually discovered as an asymptomatic mass in the third and fourth decades [1]. Few cases of intrapericardial paragangliomas associated with a tumor at the skull base have been described [2], and surgical experience is limited. We describe a case of simultaneous right atrial and aortopulmonary paragangliomas discovered after a previous resection of a right glomus vagale tumor. These tumors were resected with the aid of cardiopulmonary bypass.
A 42-year-old woman presented to her local pulmonologist with a history of dysphoria, dizziness, and anxiousness for 6 months. These symptoms worsened with exercise. On physical examination no abnormalities were noted. Laboratory test results included a normal thyroid panel, normal prolactin level, and a negative pregnancy test. Urine levels of catecholamines, metanephrines, and dopamine were within normal limits. However, the serum dopamine level was elevated. A chest radiograph was normal. A subsequent magnetic resonance image showed a pituitary mass and a 5 x 3-cm mass originating near the right carotid bifurcation and the right vagus nerve.
The removal of the tumor near the carotid bifurcation was thought to be of higher priority than removal of the pituitary tumor. This operation was performed elsewhere and was complicated by right vocal cord paralysis due to the fact that the tumor was a glomus vagale tumor arising from the right vagus nerve. Pathologic evaluation of this tumor revealed findings consistent with paraganglioma.
Six months later, serum dopamine levels were still elevated. An octreotide scan was performed and two areas lit up in the mediastinum: one near the right atrium and one in the aortopulmonary window. Magnetic resonance imaging showed a 2.5-cm mass in the aortopulmonary window (Fig 1A
) and a mass in the right atrioventricular groove (Fig 1B). However, it could not be determined whether the right atrial mass was intracardiac or extracardiac. An MIBG scan was performed, which delineated the aortopulmonary window mass, but the margins of the atrial mass could not be further defined. Magnetic resonance imaging with gadolinium revealed a 3.5-cm mass in the right atrium. The mass was noted to be surrounded by epicardial fat, but whether the mass was intracardiac or extracardiac still could not be determined. Transesophageal echocardiography was performed at yet another institution, revealing a 4 x 3.5 x 3.3-cm mass adjacent to the right atrium. The right coronary artery was displaced but not engulfed by the tumor, and no blood flow was noted within the mass. At this point the patient's care was transferred to our institution.
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Fig 1. . (A) Sagittal-view magnetic resonance image of the chest showing the aortopulmonary window tumor. (B) Sagittal-view magnetic resonance image of the chest showing intracardiac tumor.
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We performed cardiac catheterization, which showed normal coronary arteries and normal ejection fraction but a tumor blush in the right atrioventricular groove with possible right coronary artery steal phenomenon. The decision was made to perform a sternotomy to remove the tumors. The patient was placed on cardiopulmonary bypass with standard aortic and bicaval cannulation. The heart was not arrested. The lungs were deflated and the heart was retracted toward the patient's right side. The aortopulmonary window mass was in the region of the ligamentum arteriosum and was in close proximity to the left recurrent laryngeal nerve. The ligamentum was divided and the ends were oversewn, and the mass was dissected from the pericardium and the recurrent laryngeal nerve. The cardiac tumor was highly vascularized and surrounded the proximal right coronary artery. This mass was dissected free from the epicardium and the right atrium medial to the right atrial appendage. The right coronary artery was transected to remove the tumor, and a single-vessel bypass graft was made. An argon beam coagulator and fibrin glue were used to facilitate hemostasis within both tumor beds.
The patient had an uneventful course postoperatively and was discharged 4 days after the procedure with her left vocal cord intact. The pathology report expressed the findings of "zellballen" growth pattern with few mitotic features in both masses, consistent with paragangliomas. Postoperative serum dopamine levels were normal and a postoperative octreotide scan showed no uptake in the mediastinal area. Six months after her cardiac operation, the patient underwent removal of her pituitary tumor and is feeling well. Pathologic evaluation revealed a benign pituitary adenoma. Her plasma dopamine levels remain normal.
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Comment
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Paragangliomas have been classified according to anatomy by the classic description of Glenner and Grimley [3]. The two anatomic classes within the mediastinum include the aortosympathetic (associated with the posterior mediastinum) and brachiometric (associated with the anterior mediastinum and paraganglions of the coronary and aortopulmonary vessels). These tumors are rare, as only 2% of all paragangliomas are located in the chest [4], and as of 1986 only 149 cases of intrathoracic paragangliomas had been reported [5]. This case is exceedingly rare because of the synchronous appearance of multiple tumors.
The implication of multiple tumors is threefold. The first is that these lesions represent a primary tumor with metastases. This was not substantiated by pathologic investigation, which showed only a few mitotic figures in the mediastinal and glomus vagale tumors and that the pituitary tumor was a benign adenoma. Equally unlikely is the possibility that these tumors are a complex associated with the multiple endocrine syndromes. However, preoperative blood and urine studies had negative results, and pathologic investigation did not reveal pheochromocytoma. The third possibility, which we believe to be the case, is that these lesions were synchronous lesions occurring along the paraganglionic system.
The diagnosis of these tumors can be elusive. The standard diagnostic tool when a paraganglioma is suspected is the iodine 131 MIBG scan [6]. Recent emphasis on magnetic resonance imaging studies has shown good visualization when used for preoperative imaging [7] However, magnetic resonance imaging alone may not be adequate. In our case, the patient was subjected to many imaging techniques, all of which did not show the exact anatomic relationship of the tumor to the right coronary artery and the depth of the tumor into the heart. The importance of intraoperative management cannot be overstated.
A significant problem in our case was the previous removal of a right glomus vagale tumor, which left the patient with permanent right-sided vocal cord paralysis. When the tumor in the aortopulmonary window was discovered, the problem of complete vocal cord paralysis with impending tracheostomy was discussed. Keeping the left recurrent laryngeal nerve intact, therefore, was of utmost importance. Because radiation therapy is of limited usefulness in these cases [8], the only curative option was surgical removal. Fortunately, the tumor had not invaded the left recurrent laryngeal nerve and the patient recovered fully.
Our biggest concern was that of surgical approach. A left thoracotomy would have permitted resection of the aortopulmonary window tumor, but not of the right atrial tumor. Standard sternotomy without bypass may have permitted resection of the right atrial tumor but not of the deep aortopulmonary window tumor. The use of cardiopulmonary bypass permitted maximal manipulation of a decompressed heart for easy dissection of the aortopulmonary window. In addition, the right atrioventricular groove tumor was found to be subepicardial and arising from the right coronary artery, thereby necessitating a single-vessel bypass graft. Performing this resection and bypass without cardiopulmonary bypass would have been difficult. Cardiopulmonary bypass clearly permitted simultaneous resection of two difficult tumors for which no other form of therapy seemed adequate.
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Footnotes
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Address reprint requests to Dr Tribble, Department of Surgery, UVA Health Sciences Center, Box 181, Charlottesville, VA 22908.
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References
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- Glenner GG, Grimley PM. Tumors of the extra-adrenal paraganglion system. In: Firminger HI, ed. Atlas of tumor pathology, 2nd series, fasc 9. Washington DC: Armed Forces Institute of Pathology, 1974:13–60.
- Abad C, Jimenez P, Santana C, et al. Primary cardiac paraganglioma. J Cardiovasc Surg 1992;33:768–72.[Medline]
- Dunn GD, Brown MJ, Sapsford RN, et al. Functioning middle mediastinal paraganglioma associated with intercarotid paragangliomas. Lancet 1986;1:1061–4.[Medline]
- Shapiro B, Sisson J, Kalff V, et al. The location of middle mediastinal pheochromocytomas. J Thorac Cardiovasc Surg 1984;87:814–20.[Abstract]
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- Massey V, Wallner K. Treatment of metastatic chemodectoma. Cancer 1992;69:790–2.[Medline]
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Successful perioperative management of a middle mediastinal paraganglioma.
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September 1, 2006;
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705 - 706.
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Abstract
Introduction
