Last reviewed: June 2020
Last updated: December  2019



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
More 1st investigations to order

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)
More investigations to consider

Treatment algorithm


Bridget Sinnott

Associate Professor of Medicine

Medical College of Georgia




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 reviewersVIEW ALL

Clinical Endocrinologist and Research Scientist

Department of Endocrinology, Diabetes, and Metabolism

Cleveland Clinic




BAH declares that she has no competing interests.

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