Cardiac Paraganglioma: Diagnostic and Surgical Challenges

Division of Cardiovascular Medicine, Stanford University, Stanford, California 94305, USA.
Journal of Cardiac Surgery (Impact Factor: 0.89). 01/2012; 27(2):178-82. DOI: 10.1111/j.1540-8191.2011.01378.x
Source: PubMed


Primary cardiac paragangliomas are rare extra-adrenal tumors. Though they account for less than 1% of all primary cardiac tumors, they are considerable sources of morbidity and mortality. In this case review, we discuss the challenges associated with the diagnosis and management of cardiac paragangliomas.

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    • "R.A. Yes Surg. No Yes Huo et al. [30] 2012 2 Palpitation L.A.; R.A. Yes Surg. No Yes Aki et al. [31] "
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    ABSTRACT: Functioning paraganglioma is extra-adrenal catecholamine-secreting tumours that may cause secondary hypertension. Primary intrapericardial paragangliomas are very rare and are located adjacent to the great vessels or heart, typically near the left atrium. These tumours are an exceptionally uncommon finding during the investigation of refractory hypertension. However, in recent years, intrapericardial paragangliomas have been diagnosed incidentally with increased frequency, due to the extensive use of radiologic chest imaging. The mainstay of treatment of functioning intrapericardial paraganglioma is surgical removal, which usually achieves blood pressure normalization. Due to the locations of these tumours, the surgical approach is through a median sternotomy or posterolateral thoracotomy, and manipulation-induced catecholamine release may cause paroxysmal hypertension. Typically in these patients, blood pressure fluctuates dramatically intra- and post-operatively, increasing the risk of cardiovascular complications. We review here the current modalities of perioperative fluid and hypotensive drug administration in the setting of surgery for functioning intrapericardial paraganglioma and discuss the recently proposed paradigm shift that omits preoperative preparation.
    International Journal of Hypertension 02/2014; 2014:812598. DOI:10.1155/2014/812598
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    • "Cardiac paraganglionic tumors are rare neoplasms derived from the neural crest in the mediastinum, associated with the autonomic nervous system, which account for 0.3% of all mediastinal neoplasms and less than 1% of all primary cardiac tumors [6]. They arise from either the branchiomeric paraganglia (coronary or aortopulmonary) or the visceral-autonomic paraganglia (atrium or interatrial septum) and less than 50% are functional; the majority of functional tumors are intraadrenal pheochromocytomas, being uncommon at the heart. "
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    ABSTRACT: 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.
    Journal of Cardiothoracic Surgery 01/2013; 8(1):22. DOI:10.1186/1749-8090-8-22 · 1.03 Impact Factor
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    ABSTRACT: Cardiac neoplasms and other mass-forming lesions are not commonly encountered in surgical pathology practice. Fortunately, for the most part, these fall into a small group of well characterized and readily-recognized entities, although they are not without diagnostic dilemmas. A brief and practical synopsis of cardiac tumors is presented in this section with attention to more frequently encountered and clinically significant diagnostic challenges as well as pertinent clinical associations and prognostic information.
    Surgical Pathology Clinics 06/2012; 5(2):453–483. DOI:10.1016/j.path.2012.04.007
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