Last reviewed: April 2018
Last updated: November  2017



History and exam

Key diagnostic factors

  • presence of risk factors
  • headache
  • palpitations
  • diaphoresis
  • hypertension
  • hypertensive retinopathy
  • pallor
  • impaired glucose tolerance/diabetes mellitus
  • FHx endocrine disorders
  • hx prior phaeochromocytoma
  • tachyarrhythmias and myocardial infarction
  • panic attacks or a 'sense of doom'

Other diagnostic factors

  • orthostatic hypotension
  • hypercalcaemia
  • Cushing's 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
Full details

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-fluorodopamine (18F-FDA), 18F-fluorodihydroxyphenylalanine (18F-FDOPA), and 18F-fluoro-2 deoxy-D-glucose (18F-FDG) positron emission tomography (PET)
Full details

Treatment algorithm


Authors VIEW ALL

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, the previous contributor to this monograph.


SG declares that she has no competing interests.

Peer reviewers VIEW ALL

Clinical Endocrinologist and Research Scientist

University of Illinois at Chicago



BAH declares that she has no competing interests.

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