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

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 e...

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Bibliographic Details
Published in:Journal of cardiothoracic surgery 2013-01, Vol.8 (1), p.22-22, Article 22
Main Authors: González López, María Teresa, González, Sergio González, García, Esteban Sarria, Romero, Stella González, de Loma, Julio Gutiérrez
Format: Article
Language:English
Subjects:
Cardiac Surgical Procedures - methods
Cardiopulmonary Bypass
Case Report
Catecholamines - blood
Coronary artery bypass
Female
Gene mutations
Genetic aspects
Heart
Heart Atria - surgery
Heart Neoplasms - surgery
Humans
Middle Aged
Paraganglioma - blood
Paraganglioma - surgery
Reconstructive Surgical Procedures - methods
Sternotomy
Transplantation
Tumors
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Summary: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.
ISSN:1749-8090
1749-8090
DOI:10.1186/1749-8090-8-22