Pheochromocytoma – why is its early diagnosis so important for patient?

Authors: Tomáš Zelinka;  Jiří Widimský Jr
Authors‘ workplace: Centrum pro výzkum, diagnostiku a léčbu hypertenze III. interní kliniky – kliniky endokrinologie a metabolizmu 1. LF UK a VFN Praha, přednosta prof. MUDr. Štěpán Svačina, DrSc., MBA
Published in: Vnitř Lék 2015; 61(5): 487-491
Category: Reviews


Pheochromocytoma may present with various clinical signs, symptoms due to continuous and/or paroxysmal release of catecholamines. Arterial hypertension may be sustained and/or paroxysmal and palpitations are mainly due to sinus tachycardia. In some cases, even as the first manifestation of pheochromocytoma, may occur severe cardiovascular complications such as hypertensive emergency, myocardial ischemia, cardiomyopathy and heart failure, multisystem crisis or shock. Catecholamine release may be also associated with arrhythmias – tachycardias (supraventricular or ventricular) or less frequently bradycardias (AV blocks and junctional). The effect of catecholamines is not restricted to myocardium, but may also lead to cerebrovascular impairment such as transient ischemic attack or stroke. As many of these complications may be life-threatening, the only prevention is early diagnosis of pheochromocytoma and proper treatment, in particular in specialized centers.

Key words:
arrhythmias – catecholamines – cerebrovascular impairment – hypertension – myocardial ischemia – orthostatic hypotension -pheochromocytoma


1. Zelinka T, Eisenhofer G, Pacák K. Pheochromocytoma as a catecholamine producing tumor: implications for clinical practice. Stress 2007; 10(2): 195–203.

2. Neumann HP, Vortmeyer A, Schmidt D et al. Evidence of MEN-2 in the original description of classic pheochromocytoma. N Engl J Med 2007; 357(13): 1311–1315.

3. Pacák K. Feochromocytom. Galén: Praha 2008. ISBN 978–80–7262–519–2.

4. Jochmanová I, Zelinka T, Widimský J jr et al. HIF signaling pathway in pheochromocytoma and other neuroendocrine tumors. Phys Res 2014; 63(Suppl 2): S251-S262.

5. Eisenhofer G, Peitzsch M. Laboratory Evaluation of Pheochromocytoma and Paraganglioma. Clin Chem 2014; 60(12): 1486–1499.

6. Kopetschke R, Slisko M, Kilisli A et al. Frequent incidental discovery of phaeochromocytoma: data from a German cohort of 201 phaeochromocytoma. Eur J Endocrinol 2009; 161(2): 355–361.

7. Graham JB. Pheochromocytoma and hypertension; an analysis of 207 cases. Int Abstr Surg 1951; 92(2): 105–121.

8. McNeil AR, Blok BH, Koelmeyer TD et al. Phaeochromocytomas discovered during coronial autopsies in Sydney, Melbourne and Auckland. Aust NZ J Med 2000; 30(6): 648–652.

9. Zelinka T, Pacák K, Widimský J Jr. Characteristics of blood pressure in pheochromocytoma. Ann NY Acad Sci 2006; 1073: 86–93.

10. Streeten DH, Anderson GH Jr. Mechanisms of orthostatic hypotension and tachycardia in patients with pheochromocytoma. Am J Hypertens 1996; 9(8): 760–769.

11. Zelinka T, Widimský J, Weisserová J. Diminished circadian blood pressure rhythm in patients with asymptomatic normotensive pheochromocytoma. Phys Res 2001; 50(6): 631–634.

12. Zelinka T, Štrauch B, Petrák O et al. Increased blood pressure variability in pheochromocytoma compared to essential hypertension patients. J Hypertens 2005; 23(11): 2033–2039.

13. Prejbisz A, Lenders JW, Eisenhofer G et al. Cardiovascular manifestations of phaeochromocytoma. J Hypertens 2011; 29(11): 2049–2060.

14. Agarwal G, Sadacharan D, Kapoor A et al. Cardiovascular dysfunction and catecholamine cardiomyopathy in pheochromocytoma patients and their reversal following surgical cure: Results of a prospective case-control study. Surgery 2011; 150(6): 1202–1211.

15. Giavarini A, Chedid A, Bobrie G et al. Acute catecholamine cardiomyopathy in patients with phaeochromocytoma or functional paraganglioma. Heart 2013; 99(19): 1438–1444.

16. Yoshikawa T. Takotsubo cardiomyopathy, a new concept of cardiomyopathy: Clinical features and pathophysiology. Int J Cardiol 2014; 182: 297–303. Dostupné z DOI: <>.

17. Agarwal V, Kant G, Hans N et al. Takotsubo-like cardiomyopathy in pheochromocytoma. Int J Cardiol 2011; 153(3): 241–248.

18. Messerli FH, Loughlin KR, Messerli AW et al. The president and the pheochromocytoma. Am J Cardiol 2007; 99(9): 1325–1329.

19. Whitelaw BC, Prague JK, Mustafa OG et al. Phaeochromocytoma [corrected] crisis. Clin Endocrinol (Oxf) 2014; 80(1): 13–22.

20. Majic T, Aiyagari V. Cerebrovascular manifestations of pheochromocytoma and the implications of a missed diagnosis. Neurocrit Care 2008; 9(3): 378–381.

21. Stolk RF, Bakx C, Mulder J et al. Is the excess cardiovascular morbidity in pheochromocytoma related to blood pressure or to catecholamines? J Clin Endocrinol Metab 2013; 98(3): 1100–1106.

22. Zelinka T, Petrák O, Turková H et al. High incidence of cardiovascular complications in pheochromocytoma. Horm Metab Res 2012; 44(5): 379–384.

23. Holaj R, Zelinka T, Wichterle D et al. Increased carotid intima-media thickness in patients with pheochromocytoma in comparison to essential hypertension. J Hum Hypertens 2009; 23(5): 350–358.

24. Petrák O, Štrauch B, Zelinka T et al. Factors influencing arterial stiffness in pheochromocytoma and effect of adrenalectomy. Hypertens Res 2010; 33(5): 454–459.

25. Pacák K. Preoperative management of the pheochromocytoma patient. J Clin Endocrinol Metab 2007; 92(11): 4069–4079.

26. Scholten A, Cisco RM, Vriens MR et al. Pheochromocytoma crisis is not a surgical emergency. J Clin Endocrinol Metab 2013; 98(2): 581–591.

27. Manger WM. An overview of pheochromocytoma: history, current concepts, vagaries, and diagnostic challenges. Ann N Y Acad Sci 2006; 1073: 1–20.

Diabetology Endocrinology Internal medicine

Article was published in

Internal Medicine

Issue 5

2015 Issue 5

Most read in this issue

This topic is also in:

Forgotten password

Don‘t have an account?  Create new account

Forgotten password

Enter the email address that you registered with. We will send you instructions on how to set a new password.


Don‘t have an account?  Create new account