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1Department of Internal Medicine, Radboud University Medical Center, Nijmegen, The Netherlands.
2Department of Medicine III, University Hospital and Medical Faculty Carl Gustav Carus, Dresden University of Technology, Dresden, Germany.
3Institute of Clinical Chemistry and Laboratory Medicine, University Hospital and Medical Faculty Carl Gustav Carus, Dresden University of Technology, Dresden, Germany.
Copyright © 2017 Korean Endocrine Society
This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
CONFLICTS OF INTEREST: No potential conflict of interest relevant to this article was reported.
Adapted from Lenders et al. [15].
Signs or symptoms suggesting catecholamine excess, in particular if paroxysmal |
Unexpected blood pressure response to drugs, surgery, or anesthesia |
Unexplained blood pressure variability |
Incidentaloma, also in normotensive patients |
Difficult to control blood pressure |
Previous treatment for pheochromocytoma or paraganglioma |
Hereditary risk of pheochromocytoma or paraganglioma in family members |
Syndromic features relating to a pheochromocytoma-related hereditary syndrome |
Age, yr | Normetanephrine, nmol/L | Metanephrine, nmol/L | 3-Methoxytyramine, nmol/L |
---|---|---|---|
5–17 | 0.47 | 0.45 | 0.10 |
18–29 | 0.58 | 0.45 | 0.10 |
30–39 | 0.70 | 0.45 | 0.10 |
40–49 | 0.79 | 0.45 | 0.10 |
50–59 | 0.87 | 0.45 | 0.10 |
>60 | 1.05 | 0.45 | 0.10 |
Low threshold of consideration of PPGL is key for early diagnosis |
Search for clinical clues that require biochemical testing for PPGL |
Consider syndromic features related to hereditary pheochromocytoma syndrome |
Use as initial biochemical test: plasma or urinary metanephrines |
Blood sampling: preferably after at least 20 minutes of supine rest |
Consider proper pre-analytical test conditions, including use of interfering drugs |
Check creatinine excretion for completeness of 24-hour urine sampling |
Preferred assay method: use LC-MS/MS or HPLC-ED |
Use as first imaging test: CT scan; MRI reserved for specific indications |
Choice of functional imaging based on location and genetic background |
Consider genetic testing in all patients in the framework of genetic counselling |
Preoperative evaluation and medical preparation using α-adrenoceptor blockade are essential |
Annual postsurgical follow-up for at least 10 years is mandatory for all patients |
Follow-up should be lifelong in patients with an increased risk for recurrence |
Adapted from Lenders et al. [
PPGL, pheochromocytoma or paraganglioma; LC-MS/MS, liquid chromatography with tandem mass spectrometry; HPLC-ED, high pressure liquid chromatography with electrochemical detection; CT, computed tomography; MRI, magnetic resonance imaging.