ICU · Endocrine & metabolic emergencies
Pheochromocytoma Crisis
Also known as Pheochromocytoma · Paraganglioma · Catecholamine crisis · Phenoxybenzamine · Alpha before beta · Metanephrines · Metyrosine
The pheochromocytoma the crisis — the catecholamine-secreting the tumour (the adrenal the medulla / the paraganglioma) → the massive the catecholamine the surge → the severe the hypertension, the cardiomyopathy (the Takotsubo), the pulmonary the oedema, the multi-organ. The classic the triad (the headache, the sweating, the palpitations). The alpha-blockade the FIRST (the before the beta — the unopposed the alpha), the metanephrines the screen, the metyrosine, the surgical the resection (the after the 1 to 2 weeks the alpha the prep). The trigger the avoidance (the metoclopramide, the anaesthesia, the biopsy).
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Meet the patient
A 48-year-old woman with difficult-to-control hypertension arrives with thunderclap headache, drenching sweats, palpitations and crushing chest pain. Thirty minutes after IV metoclopramide for nausea her BP has surged to 223/102, with agitation, worsening headache, chest pain and a wide-complex tachycardia on the monitor; the CT abdomen shows a 5.9 cm right adrenal mass.[12][14]
This is a catecholamine storm from a pheochromocytoma, precipitated by the metoclopramide. The two questions that decide her next hour are the two that decide every pheo crisis: how do I lower the pressure without unopposed alpha vasoconstriction, and what must I never give. The answer to both is alpha-blockade first, beta only after, and avoid the dopamine antagonists, histamine releasers and indirect sympathomimetics.[1][12][13]
References22ShowHide
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- [2]Kuo EJ, Chen L, Wright JD, et al. Phenoxybenzamine is no longer the standard agent used for alpha blockade before adrenalectomy for pheochromocytoma: A national study of 552 patients Surgery, 2023.PMID 36167697
- [3]Steinsapir J, Carr AA, Prisant LM, et al. Metyrosine and pheochromocytoma Arch Intern Med, 1997.PMID 9129550
- [4]Lenders JW, Duh QY, Eisenhofer G, et al. Pheochromocytoma and paraganglioma: an endocrine society clinical practice guideline J Clin Endocrinol Metab, 2014.PMID 24893135
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- [6]Buitenwerf E, Osinga TE, Timmers HJLM, et al. Efficacy of α-Blockers on Hemodynamic Control during Pheochromocytoma Resection: A Randomized Controlled Trial J Clin Endocrinol Metab, 2020.PMID 31714582
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- [8]Han S, Suh CH, Woo S, et al. Performance of (68)Ga-DOTA-Conjugated Somatostatin Receptor-Targeting Peptide PET in Detection of Pheochromocytoma and Paraganglioma: A Systematic Review and Metaanalysis J Nucl Med, 2019.PMID 30030341
- [9]Lord MS, Augoustides JG. Perioperative management of pheochromocytoma: focus on magnesium, clevidipine, and vasopressin J Cardiothorac Vasc Anesth, 2012.PMID 22361482
- [10]Wells SA Jr, Asa SL, Dralle H, et al. Revised American Thyroid Association guidelines for the management of medullary thyroid carcinoma Thyroid, 2015.PMID 25810047
- [11]van der Zee PA, de Boer A Pheochromocytoma: a review on preoperative treatment with phenoxybenzamine or doxazosin Neth J Med, 2014.PMID 24829175
- [12]Leonard JB, Munir KM, Kim HK Metoclopramide induced pheochromocytoma crisis Am J Emerg Med, 2018.PMID 29534916
- [13]Guillemot J, Compagnon P, Cartier D, et al. Metoclopramide stimulates catecholamine- and granin-derived peptide secretion from pheochromocytoma cells through activation of serotonin type 4 (5-HT4) receptors Endocr Relat Cancer, 2009.PMID 18948374
- [14]Wannachalee T, Chunharojrith P Safety of preoperative carvedilol in a patient with recent atenolol-induced pheochromocytoma crisis and cardiomyopathy: A case report Ann Med Surg (Lond), 2020.PMID 33224491
- [15]Kong H, Zhang YX, Yin QL, et al. Efficacy and Safety of Preemptive Magnesium Sulfate Infusion during Pheochromocytoma and Paraganglioma Resection: A Randomized, Double-blind, Placebo-controlled Trial Anesthesiology, 2026.PMID 41043170
- [16]Gruber LM, Jasim S, Ducharme-Smith A, et al. The Role for Metyrosine in the Treatment of Patients With Pheochromocytoma and Paraganglioma J Clin Endocrinol Metab, 2021.PMID 33693908
- [17]Naruse M, Satoh F, Tanabe A, et al. Efficacy and safety of metyrosine in pheochromocytoma/paraganglioma: a multi-center trial in Japan Endocr J, 2018.PMID 29353821
- [18]Eisenhofer G, Walther M, Keiser HR, et al. Plasma metanephrines: a novel and cost-effective test for pheochromocytoma Braz J Med Biol Res, 2000.PMID 11004716
- [19]Zhikrivetskaya SO, Snezhkina AV, Zaretsky AR, et al. Molecular markers of paragangliomas/pheochromocytomas Oncotarget, 2017.PMID 28187001
- [20]Yao Y, Guo Y, Fan J, et al. Influence of duration of preoperative treatment with phenoxybenzamine and secretory phenotypes on perioperative hemodynamics and postoperative outcomes in pheochromocytoma and paraganglioma Front Endocrinol (Lausanne), 2023.PMID 37152936
- [21]Kassim TA, Clarke DD, Mai VQ, et al. Catecholamine-induced cardiomyopathy Endocr Pract, 2008.PMID 19158054
- [22]Ugleholdt R, Rasmussen AK, Haderslev PAH, et al. Preoperative blood pressure targets and effect on hemodynamics in pheochromocytoma and paraganglioma Endocr Connect, 2022.PMID 35358058