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The Clinical Conundrum of Corticotropin-independent Autonomous Cortisol Secretion in Patients with Bilateral Adrenal Masses

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Abstract

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.

Patients and 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.

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Authors and Affiliations

  1. Divisions of Endocrinology, Diabetes, Metabolism, Nutrition, and Internal Medicine, Mayo Clinic Rochester, 200 First Street SW, Rochester, Minnesota, 55905, USA

    William F. Young Jr., Dana Erickson, Adrian Vella, Charles F. Abboud & Paul C. Carpenter

  2. Department of Radiology, Mayo Clinic Rochester, 200 First Street SW, Rochester, Minnesota, 55905, USA

    Anthony W. Stanson

  3. Division of Gastroenterologic and General Surgery, Mayo Clinic Rochester, 200 First Street SW, Rochester, Minnesota, 55905, USA

    Geoffrey B. Thompson, Clive S. Grant, David R. Farley & Melanie L. Richards

  4. Department of Laboratory Medicine and Pathology, Mayo Clinic Rochester, 200 First Street SW, Rochester, Minnesota, 55905, USA

    J. Aidan Carney

  5. Department of Surgery, University of the Free State, Bloemfontein, South Africa

    Hendrick du Plessis

Authors
  1. William F. Young Jr.
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  2. Hendrick du Plessis
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  3. Geoffrey B. Thompson
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  4. Clive S. Grant
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  5. David R. Farley
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  6. Melanie L. Richards
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  7. Dana Erickson
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  8. Adrian Vella
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  9. Anthony W. Stanson
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  10. J. Aidan Carney
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  11. Charles F. Abboud
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  12. Paul C. Carpenter
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Corresponding author

Correspondence to William F. Young Jr..

Additional information

Presented at the Annual Meeting of the International Association of Endocrine Surgeons, Montreal, Canada, August 26–29, 2007.

J. A. Carney is an Emeritus Member of the Department of Laboratory Medicine and Pathology.

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Young, W.F., du Plessis, H., Thompson, G.B. et al. The Clinical Conundrum of Corticotropin-independent Autonomous Cortisol Secretion in Patients with Bilateral Adrenal Masses. World J Surg 32, 856–862 (2008). https://doi.org/10.1007/s00268-007-9332-8

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  • DOI: https://doi.org/10.1007/s00268-007-9332-8

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