Bilateral primary pigmented nodular adrenocortical disease--a case of report describing a rare cause of Cushing's syndrome

J Korean Med Sci. 1994 Dec;9(6):450-7. doi: 10.3346/jkms.1994.9.6.450.

Abstract

A case of Cushing's syndrome due to bilateral pigmented nodular adrenal disease in a 35-year-old male is presented. The adrenals showed multiple, black, variable sized nodules. Histologically the cells contained lipofuscin and either had a clear cytoplasm or an eosinophilic cytoplasm with a prominent nucleus. Lymphocytic infiltration and fatty metaplasia within the nodules are two of the prominent histological features. There is extreme internodular atrophy which suggests that primary pigmented nodular adrenocortical disease is a non-adrenocorticotropic hormone dependent condition. Since the disorder appears to involve primarily the cortex of both adrenals, the treatment of choice is bilateral adrenalectomy followed by steroid replacement. The characteristic clinicopathological manifestations that separate this diagnosis from other types of adrenal disease are also discussed. This is the first reported case in Korea to be documented with the pertinent clinicopathological findings.

Publication types

  • Case Reports

MeSH terms

  • Adipose Tissue / pathology
  • Adrenal Cortex / chemistry
  • Adrenal Cortex / diagnostic imaging
  • Adrenal Cortex / metabolism
  • Adrenal Cortex / pathology*
  • Adrenalectomy
  • Adult
  • Atrophy
  • Cushing Syndrome / etiology*
  • Cushing Syndrome / surgery
  • Dexamethasone
  • Furosemide
  • Humans
  • Hydrocortisone / metabolism
  • Inflammation
  • Lipofuscin / analysis*
  • Male
  • Metaplasia
  • Organelles / ultrastructure
  • Radiography
  • Ultrasonography

Substances

  • Lipofuscin
  • Furosemide
  • Dexamethasone
  • Hydrocortisone