Endocrine Pathology

, Volume 14, Issue 3, pp 193–212

Pheochromocytoma: Rediscovery as a catecholamine-metabolizing tumor

  • Graeme Eisenhofer
  • David S. Goldstein
  • Irwin J. Kopin
  • J. Richard Crout
Review

DOI: 10.1007/s12022-003-0012-4

Cite this article as:
Eisenhofer, G., Goldstein, D.S., Kopin, I.J. et al. Endocr Pathol (2003) 14: 193. doi:10.1007/s12022-003-0012-4

Abstract

Catecholamine-producing tumors are rare neoplasms derived mainly from chromaffin cells of the adrenal medulla (pheochromocytomas) or, in about 10% of cases, from paraganglia (paragangliomas). Diagnosis of these tumors relies heavily on measurements of urinary or plasma catecholamines or catecholamine metabolites. The metabolites are usually thought to be produced after release of catecholamines into the bloodstream. This, however, ignores observations of over 40 yr ago that catecholamines are metabolized within pheochromocytoma tumor cells. Development of improved methods for measurement of catecholamine metabolites, in particular, plasma concentrations of free normetanephrine and metanephrine, has reestablished the importance of intratumoral catecholamine metabolism. In patients with pheochromocytoma, over 90% of the elevations in plasma free normetanephrine and metanephrine result from metabolism of catecholamines within pheochromocytoma tumor cells. This process occurs continuously and independently of variations in catecholamine release. As a consequence, measurements of plasma concentrations and urinary outputs of normetanephrine and metanephrine provide more reliable methods for diagnosis of pheochromocytoma than measurements of the parent amines. Rediscovery of the importance of intratumoral catecholamine metabolism is leading to a reevaluation of the procedures used to diagnose pheochromocytoma. This review provides an update on the diagnosis of pheochromocytoma, with emphasis on identifying and correcting relevant misconceptions about catecholamine metabolism.

Key Words

Pheochromocytoma paraganglioma chromaffin cells adrenal glands hypertension catecholamines norepinephrine epinephrine normetanephrine metanephrine synthesis metabolism secretion 
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Copyright information

© Humana Press Inc 2003

Authors and Affiliations

  • Graeme Eisenhofer
    • 1
  • David S. Goldstein
    • 1
  • Irwin J. Kopin
    • 1
  • J. Richard Crout
    • 1
  1. 1.Clinical Neurocardiology Section, National Institute of Neurological Disorders and StrokeNational Institutes of HealthBethesda

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