The clinical conundrum of corticotropin-independent autonomous cortisol secretion in patients with bilateral adrenal masses.
BACKGROUND
Management of patients with bilateral adrenal masses and corticotropin (ACTH)-independent Cushing syndrome (CS) or subclinical CS is problematic. We report our experience with adrenal venous sampling (AVS) in the evaluation of 10 patients with bilateral masses who had ACTH-independent CS or subclinical CS.


METHODS
Ten patients (9 women, 1 man, mean age 56.4 years) with bilateral adrenal masses and ACTH-independent CS (n=3) or subclinical CS (n=7) underwent AVS. Autonomous cortisol secretion was documented in all cases with suppressed serum ACTH concentrations and lack of cortisol suppression with dexamethasone administration. Adrenal venous sampling was performed on the second day of dexamethasone administration. Cortisol and epinephrine levels were measured from each adrenal vein (AV) and from a peripheral vein (PV).


RESULTS
Mean (+/-SD) maximal diameter of the adrenal masses on computed tomography was 3.3+/-1.3 cm (range: 1.2-6.0 cm). Successful catheterization was confirmed with AV:PV epinephrine gradients. A cortisol AV:PV gradient>6.5 was consistent with a cortisol-secreting adenoma in 11 adrenal glands; 5 patients had clinically important bilateral autonomous cortisol hypersecretion, 3 had bilateral cortisol-secreting adenomas, and 2 had ACTH-independent macronodular adrenal hyperplasia. Adrenal venous sampling-guided adrenalectomy was completed in all 10 patients-2 patients had total bilateral adrenalectomy and 2 others had subtotal bilateral adrenalectomy. During a mean follow-up of 36.1 months (range: 0.7-123 months), CS or clinically important cortisol secretory autonomy did not recur.


CONCLUSIONS
Adrenal venous sampling contributed to the localization of autonomous hypercortisolism in the setting of ACTH-independent CS or subclinical CS in patients with bilateral adrenal masses.
Young
William F
WF
Division of Endocrinology, Department of Radiology, Mayo Clinic Rochester, 200 First Street SW, Rochester, Minnesota 55905, USA. Young.William@Mayo.edu
du Plessis
Hendrick
H
Thompson
Geoffrey B
GB
Grant
Clive S
CS
Farley
David R
DR
Richards
Melanie L
ML
Erickson
Dana
D
Vella
Adrian
A
Stanson
Anthony W
AW
Carney
J Aidan
JA
Abboud
Charles F
CF
Carpenter
Paul C
PC
D000306:Adrenal Cortex Neoplasms / Q000097:blood* / Q000175:diagnosis / Q000601:surgery; D000311:Adrenal Glands / Q000098:blood supply; D000315:Adrenalectomy; D018246:Adrenocortical Adenoma / Q000097:blood* / Q000175:diagnosis / Q000601:surgery; D000324:Adrenocorticotropic Hormone / Q000097:blood; D000328:Adult; D000368:Aged; D015331:Cohort Studies; D003480:Cushing Syndrome / Q000097:blood / Q000175:diagnosis* / Q000601:surgery*; D005260:Female; D006801:Humans; D006854:Hydrocortisone / Q000097:blood*; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D014680:Veins
D000324:Adrenocorticotropic Hormone; D006854:Hydrocortisone