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Anti-hypertensive treatment in pheochromocytoma and paraganglioma: current management and therapeutic features

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Abstract

Pheochromocytoma (PH) and paraganglioma (PG) are neuroendocrine neoplasms arising from chromaffin cells of the adrenal medulla and the sympathetic ganglia, respectively. Although are unusual cause of hypertension (HT) accounting for at most 0.1–0.2 % of cases, they may lead to severe and potentially lethal hypertensive crisis due to the effects of the released catecholamines. However, both PH and PG may be asymptomatic as ~30 % of subjects are normotensive or have orthostatic hypotension and in these cases the 24 h ambulatory blood pressure (BP) monitoring is an important toll to diagnose and treat HT. HT treatment may be difficult when PH or PG occurs in pregnancy or in the elderly subjects and in these cases a multidisciplinary team is required. When surgical excision is mandatory the perioperative management requires the administration of selective α1-adrenergic blocking agents (i.e., doxazosin, prazosin or terazosin) followed by a β-adrenergic blockade (i.e., propranolol, atenolol). This latter should never be started first because blockade of vasodilatory peripheral β-adrenergic receptors with unopposed α-adrenergic receptor stimulation can lead to a further elevation of BP. Although labetalol is traditionally considered the ideal agent due to its α- and β-adrenergic antagonism, experimental studies do not support its use in this clinical setting. As second regimen, the administration of vasodilators as calcium channel blockers (i.e., nicardipine, nifedipine) may be required to control BP. Oral and sublingual short-acting nifedipine are potentially dangerous in patients with hypertensive emergencies and are not recommend. The latest evidences into the diagnosis and treatment of hypertensive crisis due to PH and PG are reviewed here.

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Authors and Affiliations

  1. Department of Internal Medicine, Santa Maria della Misericordia Hospital, Viale Tre Martiri 140, 45100, Rovigo, Italy

    Alberto Mazza, Michela Armigliato & Laura Schiavon

  2. Head Service Nuclear Medicine & PET/CT Centre, Department of Nuclear Medicine, Radiology, Neuroradiology, Medical Physics, Santa Maria della Misericordia Hospital, Viale Tre Martiri 140, 45100, Rovigo, Italy

    Maria Cristina Marzola, Sotirios Chondrogiannis & Domenico Rubello

  3. Department of Internal Medicine, General Hospital of Vicenza, Vicenza, Italy

    Domenico Montemurro & Giorgio Vescovo

  4. Unit of Internal Medicine, University of Ferrara, Ferrara, Italy

    Marco Zuin

  5. Department of Neuroscience, Neurology and Neurophysiology Units, Santa Maria della Misericordia Hospital, Rovigo, Italy

    Roberta Ravenni

  6. Familial Cancer Clinic & Oncoendocrinology, Veneto Institute of Oncology, IRCCS & Department of Medicine—DIMED, University of Padova, Padua, Italy

    Giuseppe Opocher

  7. Department of Radiology, University of Southern California, Los Angeles, CA, USA

    Patrick M. Colletti

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Mazza, A., Armigliato, M., Marzola, M.C. et al. Anti-hypertensive treatment in pheochromocytoma and paraganglioma: current management and therapeutic features. Endocrine 45, 469–478 (2014). https://doi.org/10.1007/s12020-013-0007-y

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