Phaeochromocytoma

Last reviewed: 18 Apr 2022
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
More key diagnostic factors

Other diagnostic factors

  • orthostatic hypotension
  • hypercalcaemia
  • Cushing syndrome
  • diarrhoea
  • fever
  • papilloedema
  • abdominal masses
  • tremors
Other diagnostic factors

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
More risk factors

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

INITIAL

hypertensive crisis

ACUTE

without hypertensive crisis

Contributors

Authors

Bridget Sinnott, MD
Bridget Sinnott

Associate Professor of Medicine

Medical College of Georgia

Augusta

GA

Disclosures

BS declares that she has no competing interests.

Acknowledgements

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

Betul A. Hatipoglu, MD

Clinical Endocrinologist and Research Scientist

Department of Endocrinology, Diabetes, and Metabolism

Cleveland Clinic

Cleveland

OH

Disclosures

BAH declares that she has no competing interests.

  • Differentials

    • Anxiety and panic attacks
    • Essential or intractable hypertension
    • Hyperthyroidism
    More Differentials
  • Guidelines

    • NCCN clinical practice guidelines in oncology: neuroendocrine and adrenal tumors
    • European Society of Endocrinology Clinical Practice Guideline for long-term follow-up of patients operated on for a phaeochromocytoma or a paraganglioma
    More Guidelines
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