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

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.

Cardiac Paraganglioma

“…4,9,10,18 The Mayo Clinic presented their experience over 30 years with 14 patients with cardiac paragangliomas, with one mortality in the perioperative period from extensive bleeding. 16 A similar experience was reported from the Methodist/MD Anderson group with only one mortality out of their seven patients, and also due to excessive bleeding. 13 Patient 1 in this report had troublesome bleeding from the tumor bed.…”

“…The bleeding almost always arises from the tumor itself or the tumor bed after resection and rarely from the major vessels . The Mayo Clinic presented their experience over 30 years with 14 patients with cardiac paragangliomas, with one mortality in the perioperative period from extensive bleeding . A similar experience was reported from the Methodist/MD Anderson group with only one mortality out of their seven patients, and also due to excessive bleeding .…”

“…Complex surgical reconstruction techniques may be needed to achieve complete resection. [15][16][17]20,24 In both patients in this report, the wall of the left atrium in one and the right atrium in the other had to be excised. Extensive surgical maneuvers, such as transecting the inferior vena cava, superior vena cava, pulmonary artery or aortic root replacement, 19 have been reported for adequate exposure for tumor resection and/or hemostasis.…”

“…Complex surgical reconstruction techniques may be needed to achieve complete resection . In both patients in this report, the wall of the left atrium in one and the right atrium in the other had to be excised.…”

Paraganglioma Cardíaco. Reporte de un caso

“…3,4 El paraganglioma cardíaco es extremadamente raro, con una frecuencia sólo de 0,001 a 0.003%. Se presenta con mayor frecuencia en mujeres, entre la cuarta y quinta década de la vida, tiene un comportamiento incierto, pudiendo ser maligno en un 10% de los casos [6][7][8][9] .…”