Adrenal gland & paraganglia

Pheochromocytoma / paraganglioma


Topic Completed: 1 June 2017

Minor changes: 5 May 2021

Copyright: 2002-2021,, Inc.

PubMed Search: Hyperplasia paraganglia

Lan L. Gellert, M.D., Ph.D.
Page views in 2020: 372
Page views in 2021 to date: 329
Cite this page: Gellert LL. Hyperplasia-paraganglia. website. Accessed December 6th, 2021.
Definition / general
  • Usually in carotid bodies (may be due to chronic hypoxemia, Am J Pathol 1978;91:497), associated with high altitude or cardiopulmonary disease
  • Adrenal medulla occasionally affected, if so, usually bilateral, syndromic (e.g. multiple endocrine neoplasia type 2/MEN2, mutations in SDHB/succinate dehydrogenase iron-sulfur subunit), or sporadic
  • Adrenal medullary hyperplasia may be a precursor for pheochromocytoma in MEN2 syndrome (Neoplasia 2014;16:868)
  • May also affect vagal and aorticopulmonary paraganglia
Clinical features
  • From nonsymptomatic to hypertensive
Case reports
  • Adrenalectomy if symptomatic and clinically indicated
Gross description
  • Marked increase in weight, could be over 30 g
  • Diameter > 5 mm
Microscopic (histologic) description
  • Increased number of lobules, some confluent, composed of increased numbers of sustentacular cells and chief cells with hyperchromatic and mildly enlarged nuclei
Microscopic (histologic) images

Contributed by Lan L. Gellert, M.D., Ph.D.

Adrenal medullary hyperplasia

Positive stains
Differential diagnosis
  • Paraganglioma: extra-adrenal, usually > 300 mg, may have higher density of chief cells
  • Pheochromocytoma: > 1 cm, but otherwise morphologically similar to adrenal medullary hyperplasia
Board review style question #1
Which of the following differentiate adrenal medullary hyperplasia from pheochromocytoma?

  1. Cytological atypia
  2. Infiltrative border
  3. Mitosis
  4. Size
Board review style answer #1
D. Size. In clinical practice, adrenal medullary hyperplasia is defined as adrenal medullary proliferation with a size < 1 cm, while larger lesions are considered as pheochromocytoma.

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