Open AccessCase Report: Bilateral Pheochromocytoma: Clinical Characteristics and Surgical Management
Author(s) -
Soumeya Ettahiri,
Houda Ezzaki,
Tarik Souiki,
Karim Ibn Majdoub,
Imane Toughraï,
Khalid Mazaz
Publication year - 2022
Publication title -
sas journal of surgery
Language(s) - English
Resource type - Journals
ISSN - 2454-5104
DOI - 10.36347/sasjs.2022.v08i04.011
Subject(s) - pheochromocytoma , metanephrines , normetanephrine , metanephrine , medicine , multiple endocrine neoplasia type 2 , multiple endocrine neoplasia , magnetic resonance imaging , neurofibromatosis , paraganglioma , germline mutation , pathology , radiology , biology , mutation , biochemistry , gene
Objective: We would like to report the clinical presentation, surgical treatment and outcomes of this patient with bilateral pheochromocytoma. Introduction: Pheochromocytomas (PHEOs) are rare catecholamine-secreting neuroendocrine tumors of neural crest origin arising from chromaffin cells in the adrenal medulla with estimated incidence of 0.46 to 0.8 cases per 100,000 person-years [1]. Germline pathogenic variants associated with PHEO are now identified in 30-50% of these tumors [2]. Genetic syndromes can predispose to bilateral PHEO, such as multiple endocrine neoplasia (MEN) 2A, MEN 2B, von Hippel-Lindau (VHL), neurofibromatosis type 1 (NF1), as well as mutations in the genes of myc-associated factor X (MAX), transmembrane protein 127 (TMEM127), and mutations in the subunits of the succinate dehydrogenase complex (SDHx) [3]. When we suspect the pheochromocytoma clinically, we should look for biochemical confirmation, by dosing the plasma and urinary catecholamine (adrenaline, noradrenaline) and their metabolites (metanephrine and normetanephrine) [4]. This should be followed by tumor localisation using computed tomography (CT) imaging or magnetic resonance imaging (MRI) of the abdomen and pelvis that are the most commonly used methods and have similar sensitivities (90%-100%) and specificities (70%-80%) for tumor localisation [5]. Surgical resection followed by postoperative monitoring is the safest and the most effective therapeutic intervention for pheochromocytoma [6].