Pheochromocytoma – clinical manifestations, diagnosis and current perioperative management

Journal of Mind and Medical Sciences Volume 6 | Issue 2 Article 10 2019 Pheochromocytoma – clinical manifestations, diagnosis and current perioperative management Maria Manea Dragos R. Marcu Ovidiu G. Bratu Ana M. Stanescu Anca Pantea Stoian See next page for additional authors Follow this and additional works at: https://scholar.valpo.edu/jmms Part of the Biochemical Phenomena, Metabolism, and Nutrition Commons, Emergency Medicine Commons, Endocrinology, Diabetes, and Metabolism Commons, and the Medical Pathology Commons Recommended Citation Manea, Maria; Marcu, Dragos R.; Bratu, Ovidiu G.; Stanescu, Ana M.; Pantea Stoian, Anca; Gaman, Mihnea A.; and Diaconu, Camelia C. (2019) "Pheochromocytoma – clinical manifestations, diagnosis and current perioperative management," Journal of Mind and Medical Sciences: Vol. 6 : Iss. 2 , Article 10. DOI: 10.22543/7674.62.P243247 Available at: https://scholar.valpo.edu/jmms/vol6/iss2/10 This Review Article is brought to you for free and open access by ValpoScholar. It has been accepted for inclusion in Journal of Mind and Medical Sciences by an authorized administrator of ValpoScholar. For more information, please contact a ValpoScholar staff member at . Pheochromocytoma – clinical manifestations, diagnosis and current perioperative management Authors Maria Manea, Dragos R. Marcu, Ovidiu G. Bratu, Ana M. Stanescu, Anca Pantea Stoian, Mihnea A. Gaman, and Camelia C. Diaconu This review article is available in Journal of Mind and Medical Sciences: https://scholar.valpo.edu/jmms/vol6/iss2/10 https://scholar.valpo.edu/jmms/ https://proscholar.org/jmms/ ISSN: 2392-7674 J Mind Med Sci. 2019; 6(2): 243-247 doi: 10.22543/7674.62.P243247 Received for publication: June 22, 2019 Accepted: August 14, 2019 Review Pheochromocytoma – clinical manifestations, diagnosis and current perioperative management Maria Manea1, Dragos Radu Marcu1,2, Ovidiu Gabriel Bratu1,2, Ana Maria Alexandra Stanescu2, Anca Pantea Stoian2, Mihnea Alexandru Gaman2, Camelia Cristina Diaconu2,3 1 Carol Davila University Emergency Central Military Hospital, Bucharest, Romania Carol Davila University of Medicine and Pharmacy, Bucharest, Romania 3 Clinical Emergency Hospital of Bucharest, Bucharest, Romania 2 Abstract Pheochromocytoma is a neuroendocrine tumor characterized by the excessive production of catecholamines (epinephrine, norepinephrine, and dopamine). The diagnosis is suspected due to hypertensive paroxysms, associated with vegetative phenomena, due to the catecholaminergic hypersecretion. Diagnosis involves biochemical tests that reveal elevated levels of catecholamine metabolites (metanephrine and normetanephrine). Functional imaging, such as 123I-metaiodobenzylguanidine scintigraphy (123I-MIBG), has increased specificity in identifying the catecholamine-producing tumor and its metastases. The gold-standard treatment for patients with pheochromocytoma is represented by the surgical removal of the tumor. Before surgical resection, it is important to optimize blood pressure and intravascular volume in order to avoid negative hemodynamic events. Keywords  Highlights ✓ The gold-standard treatment in patients diagnosed with pheochromocytoma is the surgical removal of the tumor. Preoperative preparation is very important and its goal is to decrease the incidence of intraoperative cardiovascular complications. catecholamines, hypertensive paroxysm, metanephrine, normetanephrine, alpha-receptors blockade ✓ The incidence of morbidity and mortality associated with the surgical removal of these tumors has decreased recently, considering the careful and prompt management in the perioperative period and the advancements of the surgical technique. To cite this article: Manea M, Marcu DR, Bratu OG, Stanescu AMA, Stoian AP, Gaman MA, Diaconu CC. Pheochromocytoma – clinical manifestations, diagnosis and current perioperative management. J Mind Med Sci. 2019; 6(2): 243-247. DOI: 10.22543/7674.62.P243247 *Corresponding author: Camelia Diaconu, Clinical Emergency Hospital of Bucharest, Department of Internal Medicine, Calea Floreasca no. 8, Bucharest, Romania E-mail: Maria Manea et al. Introduction Pheochromocytoma is a neuroendocrine tumor derived from the chromaffin cells of the medullary portion of the adrenal glands, being characterized by the excessive production and release of catecholamines (adrenaline and noradrenaline) (1). Paraganglioma is also a catecholamine producing tumor, but with extra-adrenal localization (2). The most important clinical manifestation of pheochromocytoma is the hypertensive crisis due to the tumoral secretion of catecholamines (1). Surgical removal of the tumor represents the treatment of choice in order to cure and prevent cardiovascular complications (3). Discussions Epidemiology and etiology Pheochromocytoma occurs with equal frequency in men and women, and it can occur at any age (4). In 20% of the cases, pheochromocytoma has a hereditary transmission, with bilateral localization (3). 10% of the cases of pheochromocytoma are malignant, and paraganglioma occurs in 10-15% of the cases (5). A genetic component has been identified in 30% of the cases of pheochromocytoma, being associated with Von HippelLindau disease, with neurofibromatosis, and with multiple endocrine neoplasia type 1 and type II syndrome (MEN I and MEN II) (6). The etiology of pheochromocytoma is represented by increased tumoral production and the secretion of catecholamines (4). Pathophysiology The pathophysiological mechanism underlying pheochromocytoma is represented by the increased release of catecholamine hormones, secreted by the tumor, continuously or in an episodic manner. These hormones, which include epinephrine, norepinephrine, and dopamine, excessively stimulate alpha- and beta-adrenergic receptors, with all the clinical manifestations in pheochromocytoma being related to this (7). The symptomatology in pheochromocytoma is given by the predominant type of catecholaminergic secretion. Epinephrine stimulates beta-1 and beta-2 adrenergic receptors. By stimulating beta-2 adrenergic receptors, epinephrine causes vasodilation in striate muscles. Also, epinephrine has metabolic effects, stimulating hepatic glycogenolysis and gluconeogenesis, thus leading to hyperglycemia (8). Norepinephrine stimulates alpha-1, alpha-2, and beta-2 adrenergic receptors, leading to vasoconstriction and increased blood pressure. Epinephrine and norepinephrine have positive chronotropic effects through their action on beta-1 244 adrenergic receptors (9). Dopamine stimulates dopaminergic receptors D1 and D2. In cases of pheochromocytoma, dopamine concentrations are elevated, thus causing vasoconstriction and increased heart rate (10). Moreover, pheochromocytoma is characterized by the release of other substances, such as neuron-specific enolase, vasoactive intestinal peptide, or adrenocorticotropic hormone (ACTH), which cause various other clinical manifestations (...truncated)