• Defined as non-neoplastic increase in adrenal cortical cells
  
• Diffuse (62%) or nodular (20%) or hyperplasia with ectopic ACTH syndrome due to tumors (18%)
  
• Diffuse cases are usually bilateral, associated with elevated ACTH produced by pituitary gland, ACTH-producing tumor or excess corticotropin-releasing factor production and rarely due to ACTH receptor autoantibodies (as with Grave’s disease)
  
• Nodular cases usually are unrelated to ACTH production; may represent a later stage of diffuse hyperplasia, in which the lesion has evolved from ACTH-dependent to adrenal gland dependent
  
• Associated with MEN1 (not neoplastic in one case, Mod Pathol 1999;12:919; also thyroid neoplasms, leiomyomas, hepatic focal nodular hyperplasia and renal angiomyoliomas
  

• 42 year old woman with lipomatous metaplasia (Arch Pathol Lab Med 1999;123:167)
  

• Nodular variant: at least one nodule 0.5 cm in diameter, often diffusely nodular adrenal cortex which weighs 6+ grams without fat; glands have rounded edges
  

Bilateral cortical hyperplasia

Nodular

• Increased thickness of zona reticularis and fasciculata
  
• Cells in fasciculata may appear lipid depleted
  
• Atypical cells with large hyperchromatic nuclei may be present in nodules (“endocrine atypia”)
  
• Small micronodules may be present near central vein, containing lipid-laden clear cells similar to zona fasciculata
  

Diffuse hyperplasia

Lipomatous metaplasia

• Cells may be aneuploid or polyploid
  

• Abundant smooth endoplasmic reticulum, long microvilli
  

• Adenoma: single nodule, clonal
  

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