Last reviewed: 4 Feb 2021
Last updated: 10 Dec 2019
Summary
Definition
History and exam
Key diagnostic factors
- presence of risk factors
- headache
- palpitations
- diaphoresis
- hypertension
- hypertensive retinopathy
- pallor
- impaired glucose tolerance/diabetes mellitus
- family history of endocrine disorders
- history of prior phaeochromocytoma
- tachyarrhythmias and myocardial infarction
- panic attacks or a 'sense of doom'
Other diagnostic factors
- orthostatic hypotension
- hypercalcaemia
- Cushing syndrome
- diarrhoea
- fever
- papilloedema
- abdominal masses
- tremors
Risk factors
- Multiple endocrine neoplasia (MEN) syndrome type 2A and B
- Von Hippel-Lindau (VHL) disease
- neurofibromatosis type 1 (NF1)
- succinate dehydrogenase (SDH) subunit B, C, and D gene mutations
Diagnostic investigations
1st investigations to order
- 24-hour urine collection for catecholamines, metanephrines, normetanephrines, and creatinine
- serum free metanephrines and normetanephrines
- plasma catecholamines
- genetic testing
Investigations to consider
- CBC
- serum calcium
- serum potassium
- chromogranin A
- clonidine suppression test
- MRI of the abdomen and pelvis
- CT scan of the abdomen and pelvis
- I-123 metaiodobenzylguanidine (MIBG) scintigraphy
- 18F-fluoro-2 deoxy-D-glucose (18F-FDG) positron emission tomography (PET)
Treatment algorithm
Contributors
Authors

Associate Professor of Medicine
Medical College of Georgia
Augusta
GA
Disclosures
BS declares that she has no competing interests.
Dr Bridget Sinnott would like to gratefully acknowledge Dr Sidhbh Gallagher, a previous contributor to this topic. SG declares that she has no competing interests.
Peer reviewers
Clinical Endocrinologist and Research Scientist
Department of Endocrinology, Diabetes, and Metabolism
Cleveland Clinic
Cleveland
OH
Disclosures
BAH declares that she has no competing interests.
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