Cardiac Paraganglioma: Diagnostic and Surgical Challenges

Huo, Lei; Choi, Jong‐Soo; deLuna, Andres; Lee, David; Fleischmann, Dominik; Berry, Gerald J.; Deuse, T.; Haddad, François

doi:10.1111/j.1540-8191.2011.01378.x

Cited by 19 publications

(9 citation statements)

References 21 publications

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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.…”

Section: Discussionmentioning

confidence: 99%

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

López

González

García

et al. 2013

J Cardiothorac Surg

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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.

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Section: Discussionmentioning

confidence: 99%

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

López

González

García

et al. 2013

J Cardiothorac Surg

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“…They are neoplasms that may invade adjacent structures and therefore require excision or rarely even transplantation. 25 Although one-third of paragangliomas are malignant in other sites, cardiac paragangliomas that have metastasized have been only rarely documented histologically. Their histologic appearance is similar to that of extracardiac paraganglioma.…”

Section: Cardiac Paragangliomamentioning

confidence: 99%

The 2015 WHO Classification of Tumors of the Heart and Pericardium

Burke

Tavora

2016

Journal of Thoracic Oncology

222

203

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This article reviews the nomenclature of benign and malignant neoplasm of the heart and pericardium in the 4th edition of the World Health Organization's Classification, with emphasis on differences since the 3rd edition of 2004. The tumours are divided into benign, malignant, and intermediate tumors of uncertain behavior, with separate sections on germ cell tumours and tumors of the pericardium. There are important updates in the sarcoma classification, with emphasis on the most common site, the left atrium. The importance of the new genetic finding in cardiac myxomas, namely somatic mutations in the PRKAR1A gene underscores the importance of this alteration in the pathogenesis of these tumors. Challenges on the classification of each entity are discussed.

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“…6 They are classified as being either functional or non-functional and patients will present with either symptoms of catecholamine excess or mass effect on surrounding structures. 7 As is the case with this patient, they may also present from genetic screening investigations. Aside from the presence of malignant appearing tissue in areas where paraganglion tissue is usually absent, there exists no standardised criterion for the differentiation of benign from malignant PGL.…”

Section: Discussionmentioning

confidence: 60%

Resection of a functioning intrapericardial paraganglioma associated with succinate dehydrogenase B mutation

Siejka

Vittorio

Thakur

et al. 2019

SAGE Open Medical Case Reports

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Functional paragangliomas are rare neuroendocrine tumours that secrete catecholamines and are infrequently found in the mediastinum. We report a case of a young male with symptoms of catecholamine excess and a personal and family history of the paraganglioma predisposing succinate dehydrogenase subunit B mutation. The lesion had anatomical intrapericardial juxtaposition to important cardiac anatomy and posed the significant challenge of dissection at surgery. The lesion was successfully resected via sternotomy on cardiopulmonary bypass and confirmed histopathologically as paraganglioma. Intrapericardial paraganglioma is rare and treatment is difficult and time critical considering the proximity of cardiac anatomy as well as malignant potential.

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Cardiac Paraganglioma: Diagnostic and Surgical Challenges

Cited by 19 publications

References 21 publications

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

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

The 2015 WHO Classification of Tumors of the Heart and Pericardium

Resection of a functioning intrapericardial paraganglioma associated with succinate dehydrogenase B mutation

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