title: Corticotropin-independent macronodular adrenal hyperplasia: a clinicopathologic correlation.
abstract: OBJECTIVE
  To investigate the clinical presentation, laboratory findings, and pathologic characteristics of patients with corticotropin (ACTH)-independent macronodular adrenal hyperplasia.
  
  
  METHODS
  Retrospective review.
  
  
  METHODS
  Academic medical center.
  
  
  METHODS
  All patients with bilateral adrenocortical nodules associated with ACTH-independent hypercortisolism without clinicopathologic features of primary pigmented nodular adrenocortical disease with atrophic internodular adrenal cortex.
  
  
  METHODS
  Compare and contrast our findings with those previously reported; assess response to adrenalectomy.
  
  
  RESULTS
  Nine patients met the criteria for corticotropin-independent macronodular adrenal hyperplasia. All patients had biochemical evidence of Cushing syndrome, although repetitive testing was frequently required. As a result, the diagnosis was delayed from 1 to 20 years. In all patients, both the low- and high-dose dexamethasone suppression tests failed to suppress cortisol secretion. No patient had elevated ACTH levels, and following curative bilateral adrenalectomy, no patient subsequently developed Nelson syndrome, with follow-up ranging from 1 to 8.5 years. Unique histologic features were identified in all cases.
  
  
  CONCLUSIONS
  Amalgamating this series with other clinical reports plus basic research information, corticotropin-independent macronodular adrenal hyperplasia must be considered a separate and legitimate cause of Cushing syndrome.
authors.affiliation: Department of Surgery, Mayo Clinic, Scottsdale, Ariz, USA.
authors.forename: J M
authors.initials: JM
authors.lastname: Swain
authors.forename: C S
authors.initials: CS
authors.lastname: Grant
authors.forename: R T
authors.initials: RT
authors.lastname: Schlinkert
authors.forename: G B
authors.initials: GB
authors.lastname: Thompson
authors.forename: J A
authors.initials: JA
authors.lastname: vanHeerden
authors.forename: R V
authors.initials: RV
authors.lastname: Lloyd
authors.forename: W F
authors.initials: WF
authors.lastname: Young
mesh_terms: D000312:Adrenal Hyperplasia, Congenital / Q000150:complications / Q000175:diagnosis* / Q000378:metabolism / Q000473:pathology / Q000601:surgery; D000315:Adrenalectomy / Q000379:methods; D000324:Adrenocorticotropic Hormone / Q000097:blood* / Q000652:urine*; D000328:Adult; D000368:Aged; D003480:Cushing Syndrome / Q000209:etiology* / Q000378:metabolism; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D016896:Treatment Outcome
chemical_list: D000324:Adrenocorticotropic Hormone