Pheochromocytomas and Paragangliomas

Endocrinol Metab Clin North Am. 2019 Dec;48(4):727-750. doi: 10.1016/j.ecl.2019.08.006.

Abstract

Pheochromocytomas are rare neuroendocrine tumors. Extra-adrenal lesions arising from the autonomic neural ganglia are termed paraganglioma. Clinical symptoms are common between the adrenal and extra-adrenal forms and are determined by excess secretion of catecholamines. Hypertension is a critical and often dramatic feature of pheochromocytoma/paraganglioma, and its most prevalent reported symptom. However, given the rare occurrence of this cancer, in patients undergoing screening for hypertension, the prevalence ranges from 0.1% to 0.6%. Still, patients frequently come to the attention of endocrinologist when pheochromocytoma/paraganglioma is suspected as a secondary cause of hypertension. This article summarizes current clinical approaches in patients with pheochromocytoma/paraganglioma.

Keywords: Extra-adrenal pheochromocytoma; Hypertension; Neuroendocrine tumors; Paragangliomas; Pheochromocytomas.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Adrenal Gland Neoplasms* / complications
  • Adrenal Gland Neoplasms* / diagnosis
  • Adrenal Gland Neoplasms* / therapy
  • Humans
  • Hypertension* / diagnosis
  • Hypertension* / drug therapy
  • Hypertension* / etiology
  • Neuroendocrine Tumors* / complications
  • Neuroendocrine Tumors* / diagnosis
  • Neuroendocrine Tumors* / therapy
  • Paraganglioma* / complications
  • Paraganglioma* / diagnosis
  • Paraganglioma* / therapy
  • Pheochromocytoma* / complications
  • Pheochromocytoma* / diagnosis
  • Pheochromocytoma* / therapy