Intracardiac paragangliomas: surgical approach and perioperative management
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CASE REPORT
Intracardiac paragangliomas: surgical approach and perioperative management Brandon A. Guenthart1 · Winston Trope1 · Worasak Keeyapaj2 · Julianna J. Weiel3 · Amanda Edmonson1 · John W. MacArthur1 · Justin P. Annes4 · Y. Joseph Woo1 · Natalie S. Lui1 Received: 19 June 2020 / Accepted: 23 September 2020 © The Japanese Association for Thoracic Surgery 2020
Abstract Intracardiac paragangliomas most commonly arise from the left atrium and are often infiltrative and densely adherent to surrounding structures. Given their rarity, only scattered reports exist in the literature and standardized perioperative and surgical management is not well established. We describe a case of a 60-year-old woman with a mildly functioning intracardiac paraganglioma in which division of the superior vena cava improved exposure and enabled a complex limited resection. Further, we provide an overview of the diagnostic workup, perioperative medical management, surgical approach, and surveillance strategy in patients with these challenging tumors. Keywords Intracardiac paraganglioma · Cardiac tumor · Mediastinal mass · SDHC mutation
Introduction Catecholamine-secreting tumors, when found outside of the adrenal gland, are termed paragangliomas (PGLs). Of these, primary cardiac PGLs are extremely rare and more prevalent in women [1]. Functioning tumors are often discovered during the workup of persistent hypertension, palpitations, presyncope or syncope. Non-functioning tumors are typically discovered incidentally or present with compressive symptoms. Optimal treatment is complete surgical resection. 10-year survival in patients who underwent complete surgical Electronic supplementary material The online version of this article (https://doi.org/10.1007/s11748-020-01503-2) contains supplementary material, which is available to authorized users. * Natalie S. Lui [email protected] 1
Department of Cardiothoracic Surgery, Stanford University Medical Center, Falk Building, 300 Pasteur Dr., Stanford, CA 94305, USA
2
Department of Anesthesiology, Stanford University, Stanford, CA, USA
3
Department of Pathology, Stanford University, Stanford, CA, USA
4
Department of Medicine and Endocrinology, Stanford University, Stanford, CA, USA
resection is reported to be as high as 84% versus 50% in patients who underwent only a partial resection [4]. There is no role for radiotherapy in the neoadjuvant or adjuvant settings. Cardiac PGLs most commonly arise from the left atrium, and unlike pheochromocytomas in the adrenal gland, are often infiltrative and densely adherent to surrounding structures. The majority of published cases were resections via median sternotomy with cardiopulmonary bypass, although posterolateral thoracotomy incisions have also been used [5]. Complete resection requires the en bloc excision of involved structures, and the use of auto-transplantation or orthotopic heart transplantation has been described. We describe a case in which transection of the superior vena cava and left atriotomy provided excel
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