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ICU TopicsEndocrine & metabolic emergencies

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).

high22 referencesUpdated 26 July 202613 min readVerification in progress

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Pheochromocytoma crisis is a massive catecholamine surge from an adrenal-medulla (or extra-adrenal paraganglioma) tumour, presenting with the classic triad of paroxysmal headache, sweating and palpitations plus severe hypertension, and complicated by catecholamine cardiomyopathy (Takotsubo), pulmonary oedema and intracranial haemorrhage. The diagnosis rests on plasma or urine free metanephrines — not catecholamines, which are episodic and miss the tumour. The cardinal rule of treatment is alpha-blockade first, beta-blocker only after alpha is established — beta alone leaves unopposed alpha vasoconstriction and causes a catastrophic hypertensive crisis. Definitive treatment is surgical resection after blood-pressure-targeted alpha-blockade titrated over about two weeks.[1][6][20]

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]

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References22ShowHide
  1. [1]Bartikoski SR, Reschke DJ Pheochromocytoma Crisis in the Emergency Department Cureus, 2021.PMID 33833909
  2. [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. [3]Steinsapir J, Carr AA, Prisant LM, et al. Metyrosine and pheochromocytoma Arch Intern Med, 1997.PMID 9129550
  4. [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
  5. [5]Taïeb D, Wanna GB, Ahmad M, et al. Clinical consensus guideline on the management of phaeochromocytoma and paraganglioma in patients harbouring germline SDHD pathogenic variants Lancet Diabetes Endocrinol, 2023.PMID 37011647
  6. [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
  7. [7]Fassnacht M, Arlt W, Bancos I, et al. Management of adrenal incidentalomas: European Society of Endocrinology Clinical Practice Guideline in collaboration with the European Network for the Study of Adrenal Tumors Eur J Endocrinol, 2016.PMID 27390021
  8. [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. [9]Lord MS, Augoustides JG. Perioperative management of pheochromocytoma: focus on magnesium, clevidipine, and vasopressin J Cardiothorac Vasc Anesth, 2012.PMID 22361482
  10. [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. [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. [12]Leonard JB, Munir KM, Kim HK Metoclopramide induced pheochromocytoma crisis Am J Emerg Med, 2018.PMID 29534916
  13. [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. [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. [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. [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. [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. [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. [19]Zhikrivetskaya SO, Snezhkina AV, Zaretsky AR, et al. Molecular markers of paragangliomas/pheochromocytomas Oncotarget, 2017.PMID 28187001
  20. [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. [21]Kassim TA, Clarke DD, Mai VQ, et al. Catecholamine-induced cardiomyopathy Endocr Pract, 2008.PMID 19158054
  22. [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

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