Home Education AUAUniversity Education Products & Resources Pathology for Urologists Andrenal Gland Adrenocortical Hyperplasia

Adrenocortical Hyperplasia

Image A

Image B

Congenital adrenal hyperplasia

  • Results from an autosomal recessive defect in the biosynthesis of cortisol.
  • Responsible for the large majority of cases of adrenogenital syndrome developing in the first year of life.
  • Most common enzyme deficiencies are 21-hydroxylase and 11β-hydroxylase.
  • Patients may have abnormalities of sexual development, salt wasting, hypertension or adrenal insufficiency.
  • Females may have masculinization and males have precocious puberty.
  • Gross: marked thickening of adrenal with enlarged with cerebriform appearance.
  • Histology: diffuse cortical hyperplasia, particularly involving the zona reticularis, and cortical cells are lipid depleted.
  • May have tumors in testes hilus due to increase ACTH.

Image C

Image D

Acquired adrenal hyperplasia

  • Always bilateral.
  • Associated with ACTH or CRH production from pituitary and hypothalamus or from ectopic sites (e.g. small cell carcinoma).
  • Hyperaldosteronism can be associated with AAH, although most are due to adrenocortical adenoma.
  • Often diffuse, or diffuse and nodular if nodule is >1cm (image A).
  • Histology:
    • Increased thickness of zona fasciculata with lipid depletion of the cortical cells (ACTH) (image B).
    • Increased thickness of zona glomerulosa (hyperaldosteronism) (image C).