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      Influence of secretory phenotype and preoperative preparation on surgical outcome in pheochromocytoma

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          Abstract

          Objectives

          Surgery of pheochromocytomas (PCs) still carries a high risk of haemodynamic complications during the perioperative period. We aimed to evaluate the influence of their secretory phenotype and preoperative alpha-blocker treatment on surgical outcome.

          Design

          A retrospective monocentric study at a tertiary medical centre.

          Patients

          In this study, 80 consecutive patients operated by the same team for a PC between 1988 and 2018.

          Results

          Diagnosis was based on typical symptoms and signs in 58 patients, genetic testing in 12 and work-up of an adrenal incidentaloma in 9. It was made during surgery in one patient. A genetic predisposition was found in one-third of index cases (21/62). The majority of the patients (73/79) had a secreting PC; more than 2/3 had an adrenergic phenotype and less than 1/3 a noradrenergic phenotype. The rate of perioperative haemodynamic complications was not influenced by the secretory phenotype, but persistent hypertension after surgery, recurrence and malignancy were more frequently observed in patients with a noradrenergic tumour. Preoperative alpha-blocker treatment was given for ≥ 14 days in 29 patients and, although being more symptomatic at diagnosis, these patients had less haemodynamic complications (3/29 vs 12/51 non-treated patients, P = 0.05).

          Conclusions

          The occurrence of haemodynamic complications during surgery was not significantly affected by the secretory phenotype in our study, but noradrenergic tumours show a worse post-surgical outcome. Our data also provide additional support in favour of a sufficient preoperative alpha-blockade in patients with pheochromocytoma.

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          Most cited references35

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          Pheochromocytoma and paraganglioma: an endocrine society clinical practice guideline.

          The aim was to formulate clinical practice guidelines for pheochromocytoma and paraganglioma (PPGL).
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            Phaeochromocytoma.

            Phaeochromocytomas are rare neuroendocrine tumours with a highly variable clinical presentation but most commonly presenting with episodes of headaches, sweating, palpitations, and hypertension. The serious and potentially lethal cardiovascular complications of these tumours are due to the potent effects of secreted catecholamines. Biochemical testing for phaeochromocytoma is indicated not only in symptomatic patients, but also in patients with adrenal incidentalomas or identified genetic predispositions (eg, multiple endocrine neoplasia type 2, von Hippel-Lindau syndrome, neurofibromatosis type 1, and mutations of the succinate dehydrogenase genes). Imaging techniques such as CT or MRI and functional ligands such as (123)I-MIBG are used to localise biochemically proven tumours. After the use of appropriate preoperative treatment to block the effects of secreted catecholamines, laparoscopic tumour removal is the preferred procedure. If removal of phaeochromocytoma is timely, prognosis is excellent. However, prognosis is poor in patients with metastases, which especially occur in patients with large, extra-adrenal tumours.
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              Preoperative management of the pheochromocytoma patient.

              Pheochromocytomas are rare neuroendocrine tumors with a highly variable clinical presentation, but they most commonly present as spells of headaches, sweating, palpitations, and hypertension. Patients with pheochromocytoma may develop complicated and potentially lethal cardiovascular and other complications, especially in the setting of diagnostic or interventional procedures (e.g. upon induction of anesthesia or during surgery). The serious and potentially lethal nature of such complications is due to the potent effect of paroxysmal release of catecholamines. Because this warrants prompt diagnosis and treatment, the physician should be aware of the clinical manifestations and complications of catecholamine excess and be able to provide proper preoperative management to minimize catecholamine-related pre-, intra-, and postoperative adverse events. The following clinical scenario and discussion aim to enhance the knowledge of the physician regarding the behavior of pheochromocytoma and to outline current approaches to comprehensive preoperative management of patients suffering from this tumor.

                Author and article information

                Journal
                Endocr Connect
                Endocr Connect
                EC
                Endocrine Connections
                Bioscientifica Ltd (Bristol )
                2049-3614
                January 2021
                14 December 2020
                : 10
                : 1
                : 92-101
                Affiliations
                [1 ]Department of Endocrinology and Nutrition , Cliniques Universitaires Saint-Luc, Université Catholique de Louvain, Brussels, Belgium
                [2 ]Department of Cardiology , Cliniques Universitaires Saint-Luc, Université Catholique de Louvain, Brussels, Belgium
                [3 ]Department of Biology , Cliniques Universitaires Saint-Luc, Université Catholique de Louvain, Brussels, Belgium
                [4 ]Endocrine Surgery , Cliniques Universitaires Saint-Luc, Université Catholique de Louvain, Brussels, Belgium
                Author notes
                Correspondence should be addressed to D Maiter: dominique.maiter@ 123456uclouvain.be

                *(R M Furnica and M M Dusoruth contributed equally to this work)

                Article
                EC-20-0537
                10.1530/EC-20-0537
                7923042
                33320109
                4b352b47-a756-4234-b296-c431e46fcaab
                © 2021 The authors

                This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.

                History
                : 10 November 2020
                : 14 December 2020
                Categories
                Research

                pheochromocytoma,alpha-blockers,complications,surgery
                pheochromocytoma, alpha-blockers, complications, surgery

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