Aberrant cortisol regulations in bilateral macronodular adrenal hyperplasia: a frequent finding in a prospective study of 32 patients with overt or subclinical Cushing's syndrome.
Abstract
CONTEXT: ACTH-independent macronodular adrenal hyperplasia (AIMAH) is a rare and heterogeneous condition characterized by abnormal steroid production. Cortisol secretion can be regulated by aberrant hormone receptors. OBJECTIVE: A large series of patients with AIMAH were evaluated to provide information on the prevalence and profile of aberrant regulations, in relation with the functional status. DESIGN AND PATIENTS: Thirty-two consecutive patients with AIMAH were prospectively studied: 10 had a Cushing's syndrome (CS), and 22 had a subclinical CS (SCS). METHODS: A baseline endocrine evaluation was followed by an in vivo protocol in search of aberrant cortisol responses (seven provocative tests). An acute inhibition test with the somatostatin analog octreotide was also performed. RESULTS: At least one aberrant cortisol response was identified in 28 of 32 (87%) patients. The overall prevalence of aberrant responses was independent of the functional status. Responses to the upright posture and to metoclopramide were frequently observed (67 and 56% respectively). A glucagon response was frequently observed in the SCS group (58%). A cortisol inhibition by octreotide was specifically found in the three CS patients who positively responded to the mixed meal, and was observed also in 12 of 13 (92%) patients with SCS. CONCLUSIONS: Cortisol responses indicative of aberrant receptor expression were highly prevalent in AIMAH. Thorough phenotyping of AIMAH may help uncover the underlying pathophysiology.
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Receptors for several ligands are abnormally expressed at the membrane of tumoral adrenocortical cells.
“it has been shown that receptors for several ligands are abnormally expressed at the membrane of tumoral adrenocortical cells, such as those for glucose-dependent insulinotropic peptide, catecholamine, vasopressin, serotonin, angiotensin II, leptin, and LH/human chorionic gonadotropin (98 -100).”
From Diagnosis and Treatment of Subclinical Hypercortisolism (2011) · cites this paper as [100]
Somatostatin analogs have been used to control food-dependent cortisol hypersecretion due to aberrant glucose-dependent insulinotropic peptide receptors; propranolol has been proposed in adrenal hypercortisolism and leuprolide acetate in LH-dependent Cushing's syndrome.
“Indeed, somatostatin analogs have been used to control food-dependent cortisol hypersecretion due to the presence of aberrant glucose-dependent insulinotropic peptide receptors (100, 101); propranolol has been proposed in patients with adrenal hypercortisolism (102) and leuprolide acetate in LH-dependent Cushing's syndrome (103).”
From Diagnosis and Treatment of Subclinical Hypercortisolism (2011) · cites this paper as [100]
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- Authors
- Libé, R., Coste, J., Guignat, L., Tissier, F., Lefebvre, H., Barrande, G., Ajzenberg, C., Tauveron, I., Clauser, E., Dousset, B., Bertagna, X., Bertherat, J., & Groussin, L.
- Affiliations
- Centre National de la Recherche Scientifique (FR)
- Venue
- European Journal of Endocrinology
- Publisher
- Oxford University Press (OUP)
- Published
- 2010-07
- Volume
- 163
- Issue
- 1
- Pages
- 129-138
- ISSN
- 0804-4643 (ISSN-L)
- 1479-683X
- OpenAlex
- W2120059687
- Cited by
- 111
- References
- 38
- Citations by year
- 2026: 3 · 2025: 9 · 2024: 5 · 2023: 11 · 2022: 12 · 2021: 9 · 2020: 9 · 2019: 11 · 2018: 7 · 2017: 4 · 2016: 10 · 2015: 6 · 2014: 3 · 2013: 5 · 2012: 2
- FWCI
- 4.02
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- European Journal of Endocrinology (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Health Sciences › Medicine › Surgery › Adrenal and Paraganglionic Tumors
- Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Adrenal Hormones and Disorders
- Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Pituitary Gland Disorders and Treatments
- Keywords
- ACTH-independent macronodular adrenal hyperplasia, Cushing's syndrome, subclinical Cushing's syndrome, glucagon stimulation test, mixed meal tolerance test, cortisol secretion
- MeSH terms
- Adrenal Gland Diseases, Adrenal Glands, Adult, Cushing Syndrome, Female, Humans, Hydrocortisone, Hyperplasia, Male, Prospective Studies, Somatostatin, Young Adult
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