Pheochromocytoma: An approach to diagnosis

Best Pract Res Clin Endocrinol Metab. 2020 Mar;34(2):101346. doi: 10.1016/j.beem.2019.101346. Epub 2019 Oct 22.


Pheochromocytomas are rare neuroendocrine chromaffin-derived tumors that arise within the adrenal medulla. They are usually benign, but if not diagnosed or if left untreated, they can have devastating consequences. Clinical consideration of the diagnosis is paramount, as they may have protean manifestations, and a high index of suspicion is essential if serious consequences are to be avoided. An accurate biochemical diagnosis is crucial for the management of these patients: either plasma or urinary metanephrines are both highly sensitive and specific if correctly employed, but knowledge of pre- and post-analytic interference is essential. Diagnostic imaging with cross-sectional CT and/or MRI offers high sensitivity in their detection, but lack specificity. The introduction of PET/CT/MR has led to a dramatic improvement in the localization of both pheochromocytomas and paragangliomas, together with the increasing availability of new functional imaging radionuclides. Optimal investigation and accurate diagnosis is best achieved at 'centers of excellence' with expert multidisciplinary teams.

Keywords: biochemical; diagnosis; imaging; pheochromocytoma.

Publication types

  • Review

MeSH terms

  • Adrenal Gland Neoplasms / blood
  • Adrenal Gland Neoplasms / diagnosis*
  • Adrenal Gland Neoplasms / urine
  • Blood Chemical Analysis / methods
  • Blood Chemical Analysis / standards
  • Cross-Sectional Studies
  • Diagnostic Techniques, Endocrine* / standards
  • Humans
  • Magnetic Resonance Imaging
  • Metanephrine / blood
  • Metanephrine / urine
  • Pheochromocytoma / blood
  • Pheochromocytoma / diagnosis*
  • Pheochromocytoma / urine
  • Positron Emission Tomography Computed Tomography
  • Sensitivity and Specificity
  • Urinalysis / methods
  • Urinalysis / standards


  • Metanephrine