Ann Thorac Surg 2008;86:1006-1008. doi:10.1016/j.athoracsur.2008.02.071
© 2008 The Society of Thoracic Surgeons
Case Reports
Right Atrial Chemodectoma With Atypical Chest Pain: A 6-Year Surgical Follow-Up
Faranak Kargar, MD,
Mathias H. Aazami, MD*
Cardiac Surgery Department, Shaheed Rajaei Cardiovascular Medical Center, Iran University of Medical Sciences, Tehran, Iran
Accepted for publication February 21, 2008.
* Address correspondence to Dr Aazami, Cardiac Surgery Department, Shaheed Rajaei Cardiovascular Medical Center, ValiAsr Ave, Mellat Park, Tehran, Iran (Email: mathias.aazami{at}laposte.net).
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Abstract
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Paragangliomas are rare neoplasms of neural crest origin and arise from the chromaffin (pheochromocytomas) or chemoreceptor (chemodectomas) tissues. Patients with cardiac chemodectomas, hormonally inactive paragangliomas, may have chest pain or various obstructive or compressive symptoms, depending on the location of the tumor. We report the case of a right atrial chemodectoma causing atypical chest pain. The tumor was discovered at transthoracic echocardiography. Coronary angiography showed a vascular tumor fed by a branch of the circumflex artery. En bloc tumor resection was carried out under cardiopulmonary bypass, with an uneventful surgical course. After 6 years of follow-up, the patient remains asymptomatic and disease-free.
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Introduction
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Cardiac paragangliomas are rare neuroendocrine tumors that originate from the paraganglionar tissue deriving from the neural crest. They are divided into 2 subsets on the basis of their hormonal activity: pheochromocytomas and chemodectomas [1]. Chemodectomas do not cause hypercatecholaminemia, but they can give rise not only to symptoms and signs via their local mass effect or invasiveness but also to coronary steal [1]. Most previously reported cardiac paragangliomas have involved the left atrial wall. Rarely, however, they can originate in the right atrium [2, 3].
A 54-year-old woman was referred to our local cardiology department in 2001 because of a 2-week history of atypical chest pain without exertional dyspnea. There was no medical history of note, and the patient had no constitutional symptoms. Systemic blood pressure was within the normal range (120/60 mm Hg). A 12-lead electrocardiogram depicted right bundle branch block and inverted T wave in the V1-V4 leads. A chest radiograph showed a slight bulging along the right cardiac silhouette (Fig 1A). Transthoracic echocardiography revealed a large round mass (5 x 5 cm) in the right atrium (Fig 1B). The mass protruded into the tricuspid valve, but without substantial hemodynamic obstruction (mean tricuspid gradient, 2 mm Hg). Coronary angiography revealed the presence of a highly vascularized mass with arterial supply by a large branch of the circumflex artery (Fig 1C). The highly vascularized nature of the tumor raised clinical suspicion of a cardiac hemangioma or paraganglioma. Urinary catecholamine levels were within normal limits, eliminating suspicion of a catecholamine-secreting tumor. Other tumor locations were ruled out at whole-body computed tomography. An iodine 131-labeled metaiodobenzylguanidine scan yielded inconclusive findings.
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Fig 1. (A) Preoperative chest radiograph. (B) Transthoracic echocardiograph shows a large intra-atrial mass (arrow). (C) Left coronary angiogram shows the arterial supply of the tumor by a large branch of the circumflex artery (arrow) and its vascularized nature (dotted arrow). (Cx = circumflex artery; LAD = left anterior descending artery.)
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Surgery was performed via a median sternotomy using cardiopulmonary bypass, and bicaval cannulation (innominate and right femoral veins) as part of the right atrial isolation was carried out to minimize the likelihood of tumor dislodgment. The tumor's feeding artery was identified; it ran on the roof of the right pulmonary artery, which was occluded temporarily to detect any inducible ischemia or conductive disturbance. Cardiac arrest was achieved with antegrade cold-blood cardioplegia. Precaution was taken to discard blood and cardioplegia fluid returning from the coronary sinus. When the right atrium was opened, the mass was found to be a solid tumor arising from the atrial septum and deviating from the course of the superior vena cava. The tumor had a highly vascular stalk. The tumor was completely resected by ligating its stalk and excising its margin on the atrial septum. A disease-free en bloc exeresis was achieved. The resultant defect in the atrial septum was repaired using an autologous pericardial patch.
Histologic investigation documented that the tumor was highly vascular and composed of nests of cells arranged in an acinar pattern, separated by vascular septa, with moderate pleomorphism of nuclei without any mitotic activity. These are typical findings of paraganglioma (Fig 2A). After more than 6 years of follow-up, including annual transthoracic echocardiography, the patient has remained asymptomatic and free of local recurrence (Fig 2B).
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Fig 2. (A) Photomicrograph shows histologic findings typical of paragangliomas, including nests of uniform, bland polygonal cells separated by vascular septa. (B) Transthoracic echocardiogram obtained at 6-year follow-up shows the absence of local recurrence. (LA = left atrium; RA = right atrium.)
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Comment
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Primary cardiac tumors are rare, occurring in only 1% to 3% of several reported autopsy series. Approximately 75% of primary cardiac tumors are benign. Paragangliomas are unusual primary tumors of the heart, accounting for 0.3% to 1% of all mediastinal neoplasms [4, 5]. These tumors are usually discovered in the third or fourth decade of life, with a slight female predominance [1]. They are extra-adrenal tumors of the autonomic nervous system and are derived from specialized neural crest cells. Paragangliomas are divided into 2 subsets on the basis on their hormonal activity: when secreting catecholamines, they are pheochromocytomas or chromaffin-positive paragangliomas; when they are hormonally inactive, they are chemodectomas or chromaffin-negative paragangliomas [1, 3]. Fewer than 50% of paragangliomas are hormonally active, and those that are hormonally inactive occur more frequently in the pericardium [2].
Cardiac paragangliomas arise most commonly from the left atrium, followed by the interatrial septum. Other possible reported tumor sites are the anterior surface of the heart, aortopulmonary windows, aortic root, right atrium, interatrial groove, atrioventricular groove, and left ventricle [1, 4, 5]. To our knowledge, only 4 paragangliomas involving the right atrium have been reported previously [2, 3].
Paragangliomas are highly vascular tumors that can parasitize the flow of the adjacent organs. When located in the pericardium, their feeding arteries arise commonly from the left coronary tributaries, although dual vascularization from the right and left coronary systems may occur [1]. The resultant coronary steal can be a possible mechanism sustaining atypical chest pain in the setting of hormonally inactive paragangliomas, as was the case in our patient. Tamponade, superior vena caval syndrome, intracardiac flow obstruction, dyspnea, hoarseness, dry cough, hemoptysis, and dysphagia are other possible symptoms produced via the local mass effect or invasiveness of paragangliomas. Rarely, however, paragangliomas are asymptomatic and detected incidentally at cardiac imaging [1, 2]. Echocardiography is instrumental in enabling detection of cardiac tumors. Coronary angiography is of value in the workup of such highly vascularized tumors because it enables analysis of the feeding vessels of the tumor and their relation to epicardial arteries, which is of paramount relevance to operative planning [1, 2].
Paragangliomas are slow-growing tumors, and they can be locally invasive [2]. They are usually benign, and there are no reliable morphologic criteria to microscopically recognize the malignant form, the diagnosis of which is still based on the presence of metastasis [6]. Overall, 10% to 26% of thoracic paragangliomas are malignant [1, 6]. Once the tumor is located, surgical exploration with the expectation of complete resection is mandatory [1, 6, 7]. In general, resection should be undertaken with cardiopulmonary bypass, which not only confers the possibility of performing a safe resection and any unexpected additional cardiac repair but also prevents perioperative hemorrhage.
By achieving complete tumor resection with disease-free margins, good long-term survival (10 years) can be expected in 84% of patients, compared with 50% with partial resection [6, 7]. Annual evaluation of patients is the most prudent course of action because a possible rate of recurrence as high as 50% has been reported [2, 6, 8]. For nonresectable tumors, bench tumor resection with autotransplantation, radiation therapy, chemotherapy, ligation of the feeding vessels, and heart transplantation have been advocated [1].
In summary, we report a rare case of pediculated chemodectoma involving the right atrium, with atypical chest pain caused by parasitization of the coronary flow. The tumor was successfully resected en bloc with cardiopulmonary bypass. After 6 years of follow-up, the patient remains free of recurrent disease.
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References
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Abstract
Introduction
