Carmelina Gurrieri1, James J Butz2, Toby N Weingarten1, Irina Bancos3, William F Young3, Stephen D Cassivi2, Sameh M Said4, Travis J McKenzie2, David W Barbara1, Juraj Sprung5. 1. Department of Anesthesiology and Perioperative Medicine, Mayo Clinic, Rochester, Minnesota. 2. Department of Surgery, Mayo Clinic, Rochester, Minnesota. 3. Division of Endocrinology, Diabetes, Metabolism, and Nutrition, Mayo Clinic, Rochester, Minnesota. 4. Department of Cardiovascular Surgery, Mayo Clinic, Rochester, Minnesota. 5. Department of Anesthesiology and Perioperative Medicine, Mayo Clinic, Rochester, Minnesota. Electronic address: sprung.juraj@mayo.edu.
Abstract
BACKGROUND: Intrathoracic paragangliomas (PGLs) are rare tumors. Approximately 50% originate from and around cardiac structures. METHODS: A retrospective review was made of the perioperative course of patients with intrathoracic PGL resection from 2000 through 2015 at Mayo Clinic in Rochester, Minnesota. RESULTS: Twenty-two patients underwent PGL resection. Sixteen patients (73%) had functioning tumors (11, noradrenergic; 4, mixed noradrenergic and dopaminergic; 1, dopaminergic). Patients with functioning tumors received preoperative adrenergic blockade: 15 (68%), α1,2-adrenergic receptor antagonist; 4 (18%), α1-adrenergic receptor antagonists; and 13 (59%) metyrosine. Six patients with nonfunctioning tumors had no adrenergic blockade. Twelve patients had tumor resection without cardiopulmonary bypass-9 for PGL associated with the great vessels, 2 for PGL with pericardial involvement, and 1 for PGL in right atrioventricular groove. Ten patients required cardiopulmonary bypass; for 9, the tumor involved cardiac structures and for 1, it involved ascending aorta and proximal aortic arch. Of these, 1 patient had uncontrollable bleeding and died intraoperatively. Other than this single death, there were no inhospital major cardiac or pulmonary complications. Median follow-up was 8.2 years (range, 2.1 to 17.2). Six patients subsequently had metastatic disease, and of them, 1 died 6 years after the operation. CONCLUSIONS: In this series, 73% of intrathoracic PGLs were functional and involved noradrenergic, mixed noradrenergic and dopaminergic, or pure dopaminergic secretion. Cardiac and pericardial paraganglioma resection may require cardiopulmonary bypass. Although intraoperative bleeding in most complex cases may be uncontrollable, as for 1 of our patients, those who survived hospital discharge had favorable long-term outcomes.
BACKGROUND: Intrathoracic paragangliomas (PGLs) are rare tumors. Approximately 50% originate from and around cardiac structures. METHODS: A retrospective review was made of the perioperative course of patients with intrathoracic PGL resection from 2000 through 2015 at Mayo Clinic in Rochester, Minnesota. RESULTS: Twenty-two patients underwent PGL resection. Sixteen patients (73%) had functioning tumors (11, noradrenergic; 4, mixed noradrenergic and dopaminergic; 1, dopaminergic). Patients with functioning tumors received preoperative adrenergic blockade: 15 (68%), α1,2-adrenergic receptor antagonist; 4 (18%), α1-adrenergic receptor antagonists; and 13 (59%) metyrosine. Six patients with nonfunctioning tumors had no adrenergic blockade. Twelve patients had tumor resection without cardiopulmonary bypass-9 for PGL associated with the great vessels, 2 for PGL with pericardial involvement, and 1 for PGL in right atrioventricular groove. Ten patients required cardiopulmonary bypass; for 9, the tumor involved cardiac structures and for 1, it involved ascending aorta and proximal aortic arch. Of these, 1 patient had uncontrollable bleeding and died intraoperatively. Other than this single death, there were no inhospital major cardiac or pulmonary complications. Median follow-up was 8.2 years (range, 2.1 to 17.2). Six patients subsequently had metastatic disease, and of them, 1 died 6 years after the operation. CONCLUSIONS: In this series, 73% of intrathoracic PGLs were functional and involved noradrenergic, mixed noradrenergic and dopaminergic, or pure dopaminergic secretion. Cardiac and pericardial paraganglioma resection may require cardiopulmonary bypass. Although intraoperative bleeding in most complex cases may be uncontrollable, as for 1 of our patients, those who survived hospital discharge had favorable long-term outcomes.
Authors: Jwan A Naser; Martin van Zyl; Lucinda M Gruber; Rajiv Gulati; Paul A Friedman; William F Young; Pouya Hemmati; Thomas A Foley; Hartzell V Schaff; Juan A Crestanello; Sorin V Pislaru Journal: JACC Case Rep Date: 2022-07-20