Surgical excision with left atrial reconstruction of a primary functioning retrocardiac paraganglioma

Journal of Cardiothoracic Surgery

Julio Gutiérrez de Loma²  Stella González Romero¹  Esteban Sarria García²  Sergio González González²  María Teresa González López² 

[1] Endocrinology Department, Carlos Haya Regional Hospital, Málaga, Spain;Cardiovascular Surgery Department, Carlos Haya Regional Hospital, Carlos Haya Avenue, s/n. 29010, Málaga, Spain

关键词: Cardiopulmonary bypass;    Left atrium;    Cardiac tumor;    Pheochromocytoma;    Paraganglioma;   

Others  :  828806
DOI  :  10.1186/1749-8090-8-22

 received in 2012-08-01, accepted in 2013-01-11,  发布年份 2013

【 摘 要 】

About 2% of all paragangliomas are located in the chest, and a few have been described to be found in the heart. Primary cardiac paragangliomas are extremely uncommon tumors and surgical experience with this neoplasm is limited. Treatment strategies described in the literature have included simple excision, excision with reconstruction, autotransplantation after excision of the tumor and even orthotopic cardiac transplantation, depending on the extent of disease. A primary retrocardiac paraganglioma catecholamine-productive was identified in an asymptomatic 49–year old female associated to familial pheochromocytoma-paraganglioma syndrome caused by germline mutation of the gen which codifies for the subunit B of succinate dehydrogenase enzyme (SDHB). The neoplasm was surgically excised from the posterior surface of the left atrium via median sternotomy using cardiopulmonary bypass. Direct ligation of feeding vessels of the tumor along with left atrial reinforcement using a pericardial patch was performed. The post-operative course was uneventful, with normalization of catecholamine secretion and no recurrence at three-month follow-up. We review the current literature about this exceptional cardiac tumor, pathophysiological conditions and options for surgical management.

【 授权许可】

   
2013 González López et al.; licensee BioMed Central Ltd.

【 图 表 】

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【 参考文献 】

• [1]Walther MM, Keiser HR, Linehan WM: Pheochromocytoma: evaluation, diagnosis, and treatment. World J Urol 1999, 17:35-39.
• [2]Manger WM: In search of pheochromocytomas. J Clin Endocrinol Metab 2003, 88:4080-4082.
• [3]Pacheco N, Marcos G, Garcipérez FJ, Pérez C: Intrapericardial paraganglioma. Rev Esp Cardiol 2010, 63:116-117.
• [4]Elder EE, Elder G, Larsson C: Pheochromocytoma and functional paraganglioma syndrome: no longer the 10% tumor. J Surg Oncol 2005, 89:193-201.
• [5]Tomasian A, Lai C, Ruehm S, Krishnam MS: Cardiovascular magnetic resonance and PET-CT of left atrial paraganglioma. J Cardiovasc Magn Reson 2010, 12:1. BioMed Central Full Text
• [6]Huo JL, Choi JC, DeLuna A, Lee D, Fleischmann D, Berry GJ: Cardiac paraganglioma: diagnostic and surgical challenges. J Card Surg 2012, 27:178-182.
• [7]Besterman E, Bromley LL, Peart WS: An intrapericardial pheochromocytoma. Br Heart J 1974, 36:318.
• [8]Avarot DJ, Banner NR, Cantor AM: Location, localization, and surgical treatment of cardiac pheochromocytoma. Am J Cardiol 1992, 69:283-285.
• [9]Jebara VA, Uva MS, Carpentier A: Cardiac pheochromocytomas. Ann Thorac Surg 1992, 53:356-361.
• [10]Tekin UN, Khan IA, Singh N, Nair VM, Vasavada BC, Sacchi TJ: A left atrial paraganglioma patient presenting with compressive dysphagia. Can J Cardiol 2000, 16:383-385.
• [11]Cane ME, Berrizbeitia LD, Yang SS, Mahapatro D, McGrath LB: Paraganglioma of the interatrial septum. Ann Thorac Surg 1996, 61:1845-1847.
• [12]Rotker J, Oberpenning F, Scheld HH, Hertle L, Knichwitz G, Hamm D: Pheochromocytomas with extension into central vascular structures. Ann Thorac Surg 1996, 61:222-224.
• [13]Otake Y, Aoki M, Imamura N, Ishikawa M, Hashimoto K, Fujiyama R: Aortico-pulmonary paraganglioma: case report and japanese review. Jpn J Thorac Cardiovasc Surg 2006, 54:212-216.
• [14]Birkenfeld A, Bergmann M, Bräsen JH, Luft FC, Neumann H: A paraganglioma parasitizing the left circumflex coronary artery. Am J Med 2004, 116:787-788.
• [15]Khalid TJ, Zuberi O, Zuberi L, Khalid I: A rare case of cardiac paraganglioma presenting as anginal pain: case report. Cases Journal 2009, 2:72. BioMed Central Full Text
• [16]Kennelly R, Aziz R, Toner M, Young V: Right atrial paraganglioma: an unusual primary cardiac tumor. Eur J Cardiothorac Surg 2008, 33:1150-1152.
• [17]Hayek ER, Hughes MM, Speakman ED, Miller HJ, Stocker PJ: Cardiac paraganglioma presenting with acute myocardial infarction and stroke. Ann Thorac Surg 2007, 83:1882-1884.
• [18]Colwell AS, D’Cunha J, Maddaus MA: Carney’s triad paragangliomas. J Thorac Cardiovasc Surg 2001, 121:1011-1102.
• [19]Neumann HP, Bausch B, McWhinney SR, Bender BU, Gimm O, Franke G: Germ-line mutations in nonsyndromic pheochromocytoma. N Engl J Med 2002, 346:1459-1466.
• [20]Chan KM, Pontefract D, Andrews R, Naik SK: Paraganglioma of the left atrium. J Thorac Cardiovasc Surg 2001, 122:1032-1033.
• [21]Arai A, Naruse M, Naruse K, Tanabe A, Yoshimoto T, Iwama T: Cardiac malignant pheochromocytoma with bone metastases. Intern Med 1998, 37:940-944.
• [22]Neumann HP, Pawlu C, Peczkowska M, Bausch B, McWhinney SR, Muresan M: Distinct clinical features of paraganglioma syndromes associated with SDHB and SDHD gene mutations. JAMA 2004, 292:943-951.
• [23]Shao Y, Chen R, Shen ZJ, Teng Y, Huang P, Rui WB: Preoperative alpha blockade for normotensive pheochromocytoma; is it necessary? J Hypertens 2011, 29:2429-2432.
• [24]Hamilton BH, Francis IR, Gross BH: Intrapericardial paragangliomas (pheochromocytomas): imaging features. Am J Roentgenol 1997, 168:109-113.
• [25]Rakovich G, Ferraro P, Therasse E, Duranceau A: Preoperative embolization in the management of a mediastinal paraganglioma. Ann Thorac Surg 2001, 72:601-603.
• [26]Turley AJ, Hunter S, Stewart MJ: A cardiac paraganglioma presenting with atypical chest pain. Eur J Cardiothorac Surg 2005, 28:352-354.
• [27]Ceresa F, Sansone F, Rinaldi M, Patane F: Left atrial paraganglioma: diagnosis and surgical managenement. Interact Cardiovasc Thorac Surg 2010, 10:1047-1048.
• [28]Lupinski RW, Shankar S, Agasthian T, Lim CH, Mancer K: Primary cardiac paraganglioma. Ann Thorac Surg 2004, 78:43-44.
• [29]Jeevanandam V, Oz MC, Shapiro B, Barr ML, Marboe C, Rose EA: Surgical management of cardiac pheochromocytoma: resection versus transplantation. Ann Surg 1995, 221:415-419.
• [30]Brown ML, Zayas GE, Abel MD, Young WF, Schaff HV: Mediastinal paragangliomas: the mayo clinic experience. Ann Thorac Surg 2008, 86:946-951.
• [31]Pachter MR: Mediastinal nonchromaffin paraganglioma. J Thorac Cardiovasc Surg 1963, 45:152-160.
• [32]Rufini V, Treglia G, Castaldi P, Perotti G, Calcagni ML, Corsello SM: Comparison of 123-I-MIBG SPECT-CT and 18 F- DOPA PET-CT in the evaluation of patients with known or suspected recurrent paraganglioma. Nucl Med Commun 2011, 32:575-582.