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01-05-2008

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

Authors: William F. Young Jr., Hendrick du Plessis, Geoffrey B. Thompson, Clive S. Grant, David R. Farley, Melanie L. Richards, Dana Erickson, Adrian Vella, Anthony W. Stanson, J. Aidan Carney, Charles F. Abboud, Paul C. Carpenter

Published in: World Journal of Surgery | Issue 5/2008

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.

Van Heerden JA, Young WF Jr, Grant CS, et al. (1995) Adrenal surgery for hypercortisolism—surgical aspects. Surgery 117:466–472PubMedCrossRef

Young WF Jr. (2007) Clinical practice. The incidentally discovered adrenal mass. N Engl J Med 356:601–610PubMedCrossRef

Doppman JL (1993) The dilemma of bilateral adrenocortical nodularity in Conn’s and Cushing’s syndromes. Radiol Clin North Am 31:1039–1050PubMed

Koga F, Sumi S, Umeda H, et al. (1997) A case of concurrent bilateral adrenocortical adenoma causing Cushing’s syndrome. Hinyokika Kiyo 43:275–278 [in Japanese]PubMed

Makino S, Chikazawa H, Yorimitsu A, et al. (1997) A rare case of Cushing’s syndrome due to bilateral adrenocortical adenomas. Endocrinol J 44:533–540

Tamura H, Sugihara H, Minami S, et al. (1997) Cushing’s syndrome due to bilateral adrenocortical adenomas with different pathological features. Intern Med 36:804–809PubMedCrossRef

Iihara M, Suzuki R, Kawamata A, et al. (2003) Adrenal-preserving laparoscopic surgery in selected patients with bilateral adrenal tumors. Surgery 134:1066–1073PubMedCrossRef

Nomura K, Saito H, Aiba M, et al. (2003) Cushing’s syndrome due to bilateral adrenocortical adenomas with unique histological features. Endocrinol J 50:155–162

Tung SC, Wang PW, Huang TL, et al. (2004) Bilateral adrenocortical adenomas causing ACTH-independent Cushing’s syndrome at different periods: a case report and discussion of corticosteroid replacement therapy following bilateral adrenalectomy. J Endocrinol Invest 27:375–379PubMed

10.

Inoue T, Ishiguro K, Suda T, et al. (2006) Laparoscopic bilateral partial adrenalectomy for adrenocortical adenomas causing Cushing’s syndrome: report of a case. Surg Today 36:94–97PubMedCrossRef

11.

Domino JP, Chionh SB, Lomanto D, et al. (2007) Laparoscopic partial adrenalectomy for bilateral cortisol-secreting adenomas. Asian J Surg 30:154–157PubMedCrossRef

12.

Dinneen SF, Carney JA, Carpenter PC, et al. (1995) ACTH-independent Cushing’s syndrome: bilateral cortisol-producing adrenal adenomas. Endocrinol Pract 1:77–81

13.

Doppman JL, Gill JR Jr (1996) Hyperaldosteronism: sampling the adrenal veins. Radiology 198:309–312PubMed

14.

Young WF, Stanson AW, Thompson GB, et al. (2004) Role for adrenal venous sampling in primary aldosteronism. Surgery 136:1227–1235PubMedCrossRef

15.

Murphy BEP (1968) Clinical evaluation of urinary cortisol determinations by competitive protein-binding radioassay. J Clin Endocrinol Metab 28:343–348PubMed

16.

Moyer TP, Jiang NS, Tyce GM, et al. (1979) Analysis for urinary catecholamines by liquid chromatography with amperometric detection: methodology and clinical interpretation of results. Clin Chem 25:256–263PubMed

17.

Newell-Price J, Bertagna X, Grossman AB, et al. (2006) Cushing’s syndrome. Lancet 367:1605–1617PubMedCrossRef

18.

Arnaldi G, Angeli A, Atkinson AB, et al. (2003) Diagnosis and complications of Cushing’s syndrome: a consensus statement. J Clin Endocrinol Metab 88:5593–5602PubMedCrossRef

19.

Raff H, Findling JW (2003) A physiologic approach to diagnosis of the Cushing syndrome. Ann Intern Med 138:980–991PubMed

20.

Lacroix A, Bourdeau I (2005) Bilateral adrenal Cushing’s syndrome: macronodular adrenal hyperplasia and primary pigmented nodular adrenocortical disease. Endocrinol Metab Clin North Am 34:441–458PubMedCrossRef

21.

Doppman JL, Nieman LK, Travis WD, et al. (1991) CT and MR imaging of massive macronodular adrenocortical disease: a rare cause of autonomous primary adrenal hypercortisolism. J Comput Assist Tomogr 15:773–779PubMedCrossRef

22.

Carney JA, Young WF Jr (1992) Primary pigmented nodular adrenocortical disease and its associated conditions. Endocrinologist 2:6–21CrossRef

23.

Sarkar SD, Cohen EL, Beierwaltes WH, et al. (1977) A new and superior adrenal imaging agent, ¹³¹I-6beta-iodomethyl-19-nor-cholesterol (NP-59): evaluation in humans. J Clin Endocrinol Metab 45:353–362PubMedCrossRef

24.

Yu KC, Fraker DL, Ziessman HA (1996) Atlas of iodocholesterol scintigraphy (NP-59) in Cushing’s syndrome with CT and MR correlation. Clin Nucl Med 21:136–141PubMedCrossRef

25.

Lumachi F, Zucchetta P, Marzola MC, et al. (2002) Usefulness of CT scan, MRI and radiocholesterol scintigraphy for adrenal imaging in Cushing’s syndrome. Nucl Med Commun 23:469–473PubMedCrossRef

Title: The Clinical Conundrum of Corticotropin-independent Autonomous Cortisol Secretion in Patients with Bilateral Adrenal Masses
Authors: William F. Young Jr.
Hendrick du Plessis
Geoffrey B. Thompson
Clive S. Grant
David R. Farley
Melanie L. Richards
Dana Erickson
Adrian Vella
Anthony W. Stanson
J. Aidan Carney
Charles F. Abboud
Paul C. Carpenter
Publication date: 01-05-2008
Publisher: Springer-Verlag
Published in: World Journal of Surgery / Issue 5/2008
Print ISSN: 0364-2313
Electronic ISSN: 1432-2323
DOI: https://doi.org/10.1007/s00268-007-9332-8

At a glance: The STEP trials

Springer Medicine

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

Abstract

Background

Patients and Methods

Results

Conclusions

At a glance: The STEP trials

Springer Medicine

Abstract

Background

Patients and Methods

Results

Conclusions

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