Adrenal gland and paraganglia
Adrenal medulla
Adrenal medullary hyperplasia

Author: Pallav Gupta, M.D. (see Authors page)

Revised: 19 January 2016, last major update November 2013

Copyright: (c) 2002-2016, PathologyOutlines.com, Inc.

PubMed Search: Adrenal medullary hyperplasia [title]

Definition / General
  • Increase in mass of adrenal medullary cells and expansion into areas of gland where not normally present, such as tail
Etiology
  • Sporadic cases associated with cystic fibrosis, sudden infant death syndrome, nonfamilial Beckwith-Wiedemann syndrome
  • Familial cases associated with MEN 2a, 2b/3, von Hippel-Lindau disease, neurofibromatosis type 1
Clinical Features
  • May cause hypertensive symptoms similar to pheochromocytoma, Cushing Syndrome
  • Bilateral; either nodular or diffuse
  • Note: nodular hyperplasia in MEN 2a or 2b patients may act similar to pheochromocytoma
Diagnosis
  • Based on morphometry (medullary volume > 10% of gland)
Laboratory
  • Increased urinary levels of catecholamines
Radiology Description
  • On ultrasound, bilateral adrenal medullary hyperplasia is seen as a highly echogenic linear structure
  • On CT, seen as high-density linear structure (Crit Rev Diagn Imaging 1992;33:437)
Case Reports
Treatment
Clinical Images

Abnormally intense focal uptake

Gross Description
  • Familial cases usually have multiple, unencapsulated, gray-tan nodules in both glands
Gross Images

Left adrenal gland

Micro Description
  • Alveolar, trabecular or solid patterns of medullary cells with variable size and shape
  • Often medullary tissue in alar and tail regions of gland
Micro Images

No discreet tumor nodules

Positive Stains
  • Chromogranin, synaptophysin, NSE
Negative Stains
  • Inhibin
Electron Microscopy Description
  • Resembles normal medullary cells
Molecular / Cytogenetics Description
  • May be monoclonal
Differential Diagnosis
  • Cortical atrophy: makes medulla appear prominent
  • Pheochromocytoma: usually > 1 cm; increased urinary excretion of epinephrine, norepinephrine, VMA and metanephrines