Corticotropin-independent macronodular adrenal hyperplasia: a clinicopathologic correlation.
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.
Swain
J M
JM
Department of Surgery, Mayo Clinic, Scottsdale, Ariz, USA.
Grant
C S
CS
Schlinkert
R T
RT
Thompson
G B
GB
vanHeerden
J A
JA
Lloyd
R V
RV
Young
W F
WF
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
D000324:Adrenocorticotropic Hormone