Interact CardioVasc Thorac Surg 2007;6:830-831. doi:10.1510/icvts.2007.161737
© 2007 European Association of Cardio-Thoracic Surgery
Case report - Cardiac general |
Aortic paraganglioma requiring resection and replacement of the aortic root
Sai Yendamuria,
Mohammed Elfarb,
Jon-Cecil Walkesb and
Michael J. Reardonb,*
a University of Texas MD Anderson Cancer Center, Houston, TX, USA
b The Methodist Hospital, Houston, TX, USA
Received 28 June 2007;
received in revised form 28 August 2007;
accepted 29 August 2007
*Corresponding author. 6560 Fannin St, #1006, Houston, TX 77030, USA. Tel.: +1 713 793 7409; fax: +1 713 793 7428.
E-mail address: mreardon{at}tmh.tmc.edu (M.J. Reardon).
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Abstract
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This case report demonstrates the resection of an aortic root paraganglioma incidentally discovered in a 32-year-old male with replacement of the root for an oncologically complete resection.
Key Words: Paraganglioma; Cardiac tumor; Aortic root
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1. Introduction
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Cardiac paragangliomas are rare entities, constituting <5% of all cardiac tumors. These tumors typically occur in young individuals and tend to remain asymptomatic until discovered incidentally or grow to a size large enough to cause symptoms. Resection is often technically demanding due to their location. We present one such case of aortic root paraganglioma that required an aortic root replacement.
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2. Clinical summary
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A 32-year-old male was admitted to another hospital after a motorcycle accident with clavicular fracture. As part of the trauma workup, a CT-scan of the chest was performed that revealed a cardiac tumor that involved the aortic root (Fig. 2). A cardiac catheterization was performed which revealed a tumor blush from both the right and left coronary arteries (Fig. 1a,b). A percutaneous biopsy of the mass was performed that revealed a diagnosis of paraganglioma. The patient had significant intrapericardial hemorrhage from the biopsy that was managed with a pericardial drain and resolved without any further intervention. The patient was referred to our institution for further management. An echocardiogram was performed which revealed an aortic annulus of 24 mm and the appropriate sized aortic homograft was ordered. The choice of using a homograft was based on preoperative discussions with the patient regarding the use of postoperative anticoagulation. The patient had a very active outdoor life that he wanted to preserve, which would place him at a high risk for anticoagulation.
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Fig. 1. (a) Coronary angiogram: right coronary artery showing tumor blush. (b) Coronary angiogram: left coronary artery. (c) Operative view showing the location of the tumor. (d) Resected aortic body paraganglioma.
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A median sternotomy was performed. Cardiopulmonary bypass was initiated with distal ascending aortic cannulation and bicaval venous cannulation. Antegrade cardioplegia through the distal ascending aorta and retrograde cardioplegia via a catheter in the coronary sinus were given. A sump catheter was placed in the left atrium through a purse-string suture in the right superior pulmonary vein. The thymus and mediastinal lymph nodes were excised. The tumor was dissected away from the distal ascending aorta. One centimeter beyond the sinotubular junction the tumor was firmly adherent to the aortic wall, suggesting invasion, the aorta was transected at this point (Fig. 1c). The tumor extended around the base of the aorta, extending posterior to the junction of the right main pulmonary artery (PA) and the main pulmonary artery. In order to better expose this area, the PA was transected just proximal to its bifurcation. The tumor was then dissected off the PA and the left mainstem bronchus. This maneuver also facilitated the dissection of the left main coronary artery, which was mobilized as an ostial button. The right coronary ostium was also mobilized as a button. The aortic valve leaflets and the non-coronary sinus of valsalva was excised, which enabled the complete excision of the tumor with the attached aortic wall (Fig. 1d). A 24-mm aortic homograft was used to replace the aortic root with re-implantation of the coronary ostial buttons. The postoperative course was uneventful with the patient discharged to home on postoperative day 7.
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3. Discussion
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Paragangliomas are tumors arising from extra-adrenal chromaffin tissue. These have been classified based on histology, location and innervation into brachiomeric, intravagal aortico-sympathetic and visceral-autonomic groups. Aortic body paragangliomas are included in the brachiomeric group [1]. This distinction is important as the brachiomeric group typically has fewer metastases and presumably better prognosis, justifying a more aggressive resection. While most patients with aortic body paragangliomas are asymptomatic, some patients present with angina or myocardial infarction due to compromise of the coronary circulation [2, 3]. The mainstay of therapy for these tumors is complete surgical resection [4].
The case that we have presented here highlights several issues in the management of these tumors. First, a high index of suspicion is important for diagnosis as these tumors are often misdiagnosed clinically as lymphomas or cardiac sarcomas. Second, these tumors are very vascular and percutaneous biopsy can lead to life threatening hemorrhage. Therefore, open biopsy with a plan to resect is a safer approach. Preoperative embolization to decrease the vascularity of this tumor has also been described [5]. Third, proper preoperative planning is crucial as resection can sometimes be technically challenging. As we have described elsewhere, resection may entail complex maneuvers, including cardiac auto-transplantation [6]. In this case, preparations were made for replacement of the aortic root, including having available an appropriate sized aortic homograft.
We conclude that cardiac paragangliomas of the aortic root can be safely resected using well established surgical techniques with appropriate surgical planning.
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Acknowledgements
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The authors acknowledge the assistance of Shanda H. Blackmon M.D, M.P.H, Assistant Professor, Department of Surgery, Methodist Hospital, Houston, in the preparation and critical review of this manuscript.
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References
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Abstract
1. Introduction