Robotic Adrenalectomy for Pheochromocytoma in a Patient with Fontan Physiology

Abstract

PHEOCHROMOCYTOMAS ARE rare neuroendocrine tumors that arise from both chromaffin cells in the adrenal medulla and sympathetic ganglion, or nonchromaffin cells in the parasympathetic ganglion. 1 Zhao B Zhou Y Zhao Y et al. Co-occurrence of pheochromocytoma-paraganglioma and cyanotic congenital heart disease: A case report and literature review. Front Endocrinol (Lausanne). 2018; 9: 165 Crossref PubMed Scopus (6) Google Scholar Pheochromocytomas arising from sympathetic tissue more commonly secrete catecholamines than those arising outside sympathetic tissue. 2 Lenders JW Eisenhofer G Mannelli M et al. Phaeochromocytoma. Lancet. 2005; 366: 665-675 Abstract Full Text Full Text PDF PubMed Scopus (1195) Google Scholar A strong association exists between cyanotic congenital heart disease and pheochromocytomas, with a higher- than- normal incidence seen in patients with cyanotic congenital heart disease (incidence reported as high as 2.5%) versus the normal hypertensive population prevalence of 0.2% to 0.6%. 1 Zhao B Zhou Y Zhao Y et al. Co-occurrence of pheochromocytoma-paraganglioma and cyanotic congenital heart disease: A case report and literature review. Front Endocrinol (Lausanne). 2018; 9: 165 Crossref PubMed Scopus (6) Google Scholar ,3 Lenders JW Duh QY Eisenhofer G et al. Pheochromocytoma and paraganglioma: An endocrine society clinical practice guideline. J Clin Endocrinol Metab. 2014; 99: 1915-1942 Crossref PubMed Scopus (1195) Google Scholar Chronic hypoxia has been speculated as a likely pathogenic mechanism related to abnormal activation of the hypoxic pathways located within the chromaffin cells. 4 Opotowsky AR Moko LE Ginns J et al. Pheochromocytoma and paraganglioma in cyanotic congenital heart disease. J Clin Endocrinol Metab. 2015; 100: 1325-1334 Crossref PubMed Scopus (43) Google Scholar , 5 Jochmanova I Yang C Zhuang Z et al. Hypoxia-inducible factor signaling in pheochromocytoma: Turning the rudder in the right direction. J Natl Cancer Inst. 2013; 105: 1270-1283 Crossref PubMed Scopus (111) Google Scholar , 6 Favier J. Pheochromocytomas: The (pseudo)-hypoxia hypothesis. Best Practice Res Clin Endocrinol Metab. 2010; 24: 957-968 Crossref PubMed Scopus (76) Google Scholar Although Fontan is intended to mitigate cyanosis, patients may be exposed to cyanosis before Fontan repair, and patients with failing Fontan physiology may become cyanotic due to fenestration, baffle leaks, and veno-venous collateral circulation. 7 Eagle SS Daves SM. The adult with Fontan physiology: Systematic approach to perioperative management for noncardiac surgery. J Cardiothorac Vasc Anesth. 2011; 25: 320-334 Abstract Full Text Full Text PDF PubMed Scopus (24) Google Scholar Further, excess catecholamines secreted by the pheochromocytoma can mimic and induce heart failure and arrhythmias in all patients, particularly those with Fontan physiology.