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Surgical Treatment of Cardiac Pheochromocytoma: A Case Report

2009; Elsevier BV; Volume: 88; Issue: 1 Linguagem: Inglês

10.1016/j.athoracsur.2008.11.029

1552-6259

Jian Zhou, Haitao Chen, Jie Xiang, Xiao-Hong Qu, Yao-Quan Zhou, Wang‐Fu Zang,

Cancer, Hypoxia, and Metabolism

Primary cardiac pheochromocytoma is an extremely rare neoplasm. We report a 15-year-old girl who was presented with paroxysmal hypertension. An iodine-131 metaiodobenzylguanidine scintigraphy scanning showed a pheochromocytoma in her right atrial and ventricular wall. The tumor was subsequently confirmed by magnetic resonance imaging and coronary angiogram. This patient underwent a successful surgical resection of the tumor, a reconstruction of the atrial ventricular wall and right coronary artery bypass grafting. Her blood pressure remained normal thereafter. A follow-up coronary angiogram revealed a patent saphenous vein graft 4 months after the operation. Primary cardiac pheochromocytoma is an extremely rare neoplasm. We report a 15-year-old girl who was presented with paroxysmal hypertension. An iodine-131 metaiodobenzylguanidine scintigraphy scanning showed a pheochromocytoma in her right atrial and ventricular wall. The tumor was subsequently confirmed by magnetic resonance imaging and coronary angiogram. This patient underwent a successful surgical resection of the tumor, a reconstruction of the atrial ventricular wall and right coronary artery bypass grafting. Her blood pressure remained normal thereafter. A follow-up coronary angiogram revealed a patent saphenous vein graft 4 months after the operation. Pheochromocytomas are tumors originated from the stem cells of the neural crest [1Vander Salm T.J. Unusual primary tumors of the heart.Semin Thorac Cardiovasc Surg. 2000; 12: 89-100Abstract Full Text PDF PubMed Scopus (208) Google Scholar], including pheochromocytoma, which is known to release catecholamine. Among all reported pheochromocytoma cases, the incidence of extra-adrenal locations was less than 10%. However, less than 2% of them were found in the chest [2Van Heerden J.A. Sheps S.G. Hamberger B. et al.Phaeochromocytoma: current status and changing trends.Surgery. 1982; 91: 367-373PubMed Google Scholar]. The intracardiac pheochromocytoma is extremely rare. Hence, surgical treatment of a cardiac pheochromocytoma requires accurate preoperative investigation. In addition to radiologic studies, a coronary angiogram is always necessary to show the blood supply of the tumor. We believe that a successful resection of cardiac pheochromocytoma with a reconstruction of atrial ventricular wall and a combined coronary artery bypass grafting have not been previously mentioned in the literature. A 15-year-old girl was referred for paroxysmal hypertension with a history of sweating and post-exercise vomiting for more than 10 years. The patient had no headache, dizziness, and palpitations. She has no family history of pheochromocytoma and other related disorders. Her blood pressure was 200/120 mm Hg during her physical examination. Pheochromocytoma was suspected due to her significantly elevated urinary catecholamine levels, namely noradrenaline (2,163.6 μg/24-hour; normal values, 7 to 65μg/24-hour) and adrenaline (8,097 μg/24-hour; normal values, < 22 μg/24-hour). In addition, her serum nor-metanephrine level was 4,044.6 pg/mL (normal 19 to 121 pg/mL), whereas the value of metanephrine was only 78.5 pg/mL (normal, 14 to 90 pg/mL). An iodine-131 metaiodobenzylguanidine scintigraphy was performed, which showed an area of intense uptake in the middle mediastinum, but not in the adrenal glands or elsewhere (Fig 1). Magnetic resonance imaging of the chest confirmed a 5.0 × 4.0 cm2 mass invading both the right atrium and right ventricle (Fig 1). Coronary angiography demonstrated the mass was fed by the right coronary artery, which was entirely enclosed by the mass (Fig 1). The patient's blood pressure was controlled to 160/90 mm Hg and her heart rate was 86 beats/minute after preoperative treatment with doxazosin and metoprolol. A median sternotomy in the supine position was performed. The tumor was found within the right atrium with partial attachment to the anterior wall of the right ventricle (Fig 2). Under systemic hypothermia at 30°C, a round solid pheochromocytoma was excised, which was measured 6.0 × 4.9 × 4.0 cm3 and weighted 41.7 gm (Fig 2). As a segment of the right coronary artery was inside the tumor, revascularization using a saphenous vein graft was carried out. In addition, both the right atrium reconstruction and the right ventricle reinforcement were performed by an autologous pericardial patch (Fig 2). On postoperative day 1, the patient's serum nor-metanephrine level decreased to 133.8 pg/mL. Her urinary noradrenaline level dropped dramatically to the normal range (64.2 μg/24-hour). Her blood pressure remained normal since the operation. Postoperative echocardiogram showed a normal cardiac function. The patient had an uneventful recovery and was discharged home on postoperative day 17. The resected tumor was confirmed to be a right heart pheochromocytoma by histopathologic examination (Fig 3). The patient was in an excellent condition with normal blood pressure requiring no further anti-hypertensive medications at the follow-up after 4 months of operation. A repeated coronary angiogram revealed a nicely patent saphenous vein graft (Fig 4). Functionally active paragangliomas are classified as pheochromocytomas. They are closely related and originated in the ganglia of the sympathetic nervous system. The majority of Pheochromocytomas were located in adrenal glands. Less than 2% of the cases were reported to be inside the thoracic cavity. However, the pheochromocytoma with cardiac origin is even rare [1Vander Salm T.J. Unusual primary tumors of the heart.Semin Thorac Cardiovasc Surg. 2000; 12: 89-100Abstract Full Text PDF PubMed Scopus (208) Google Scholar]. Cardiac pheochromocytoma can be found on the roof of the left atrium, intra-atrial septum and the anterior surface of the heart [3Shapiro B. Sisson J. The localization of middle mediastinal pheochromocytomas.J Thorac Cardiovas Surg. 1984; 87: 814Google Scholar]. Those involving both the atrium and ventricle had never been reported in the literature. The first case of surgically resected cardiac pheochromocytoma was described in 1974 [4Wilson A.C. Bennett R.C. Niall J.F. Clarebrough J.K. Doyle A.E. Louis W.J. An unusual case of intrathoracic pheochromocytoma.Aust N Z J Surg. 1974; 44: 27-32Crossref PubMed Scopus (27) Google Scholar]. Afterward, less than 50 cases of cardiac pheochromocytomas were reported, which presented a progressively augmented surgical success in the following decades (Fig 5). The strategy of surgical treatment of pheochromocytoma relies heavily on identifying its blood supply. Total body iodine-131 metaiodobenzylguanidine scintigraphy scanning is a powerful tool to detect the tumor [5Quint L.E. Glazer G.M. Francis I.R. et al.Phaeochromocytoma and paraganglioma: comparison of MR imaging with CT and I-131 MIBG scintigraphy.Radiology. 1987; 165: 89-93Crossref PubMed Scopus (173) Google Scholar]. In this case the correct diagnosis was also confirmed by magnetic resonance imaging. In addition, the coronary angiogram was very helpful in detecting the feeding vessels of the tumor. Cardiac pheochromocytoma can be excised using cardiopulmonary bypass [6Moorjani N. Kuo J. Wilkins D. Left atrial phaeochromocytoma.Heart. 2004; 90: e64Crossref PubMed Scopus (6) Google Scholar]. In this case it also allowed a safe revascularization of the right coronary artery. In short, primary cardiac pheochromocytoma represents one of the highly active catecholamine-secreting tumors. Despite the technical challenges, surgical treatment is indeed a feasible and preferred first-line option. Overall, careful preoperative investigations and accurate information are essential to a curative surgical outcome.