Adrenocortical hyperplasia: A multifaceted disease
Abstract
Adrenocortical hyperplasia may develop in different contexts. Primary adrenal hyperplasia may be secondary to primary bilateral macronodular adrenocortical hyperplasia (PBMAH) or micronodular bilateral adrenal hyperplasia (MiBAH) which may be divided in primary pigmented nodular adrenocortical disease (PPNAD) and isolated micronodular adrenocortical disease (i-MAD). Both lead to oversecretion of cortisol and potentially to Cushing's syndrome. Moreover, adrenocortical hyperplasia may be secondary to longstanding ACTH stimulation in ACTH oversecretion as in Cushing's disease, ectopic ACTH secretion or glucocorticoid resistance syndrome and congenital adrenal hyperplasia secondary to various enzymatic defects within the cortex. Finally, idiopathic bilateral adrenal hyperplasia is the most common cause of primary aldosteronism. We will discuss recent findings on the multifaceted forms of adrenocortical hyperplasia.
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Bilateral adrenal hyperplasia, and to a lesser extent adrenal adenomas, is often associated with eutopic or ectopic hormone receptor expression with coupling to steroidogenesis.
“Bilateral adrenal hyperplasia, and to a lesser extent adrenal adenomas, is often associated with eutopic or ectopic hormone receptor expression with coupling to steroidogenesis (85).”
From Approach to the Patient: Diagnosis of Cushing Syndrome (2022) · cites this paper as [85]
Screening for aberrant hormone receptor expression with specific stimulation tests may offer an option for medical therapy via blockade of the receptor or inhibition of secretion of the endogenous ligand.
“Screening for aberrant hormone receptor expression with specific stimulation tests may offer an option for medical therapy via blockade of the receptor or inhibition of secretion of the endogenous ligand (85).”
From Approach to the Patient: Diagnosis of Cushing Syndrome (2022) · cites this paper as [85]
Examples of aberrant hormone receptors in bilateral adrenal hyperplasia include the vasopressin receptor, LH receptor, and glucose-dependent insulinotropic polypeptide receptor.
“Examples are the vasopressin receptor, LH receptor, and the glucose-dependent insulinotropic polypeptide receptor.”
From Approach to the Patient: Diagnosis of Cushing Syndrome (2022) · cites this paper as [85]
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Overview of the 2022 WHO Classification of Adrenal Cortical Tumors
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Self-citationAdrenal Cushing’s syndrome in children
Guarnotta, V., Emanuele, F., Salzillo, R., & Giordano, C. S. D. S. (2023) · Frontiers in Endocrinology · doi:10.3389/fendo.2023.1329082 · Cited by 15 · see citing works
Histopathologic Features of Adrenal Cortical Carcinoma
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Approach of Heterogeneous Spectrum Involving 3beta-Hydroxysteroid Dehydrogenase 2 Deficiency
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Primary bilateral macronodular adrenal hyperplasia: A series of 32 cases and literature review
Castro, M. A., & Reincke, M. (2023) · Endocrinología Diabetes y Nutrición (English ed ) · doi:10.1016/j.endien.2023.04.005 · Cited by 8 · see citing works
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Vaduva, P., & Bertherat, J. Y. (2024) · HORMONES · doi:10.1007/s42000-024-00608-0 · Cited by 5 · see citing works
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Delivanis, D. A., Vassiliadi, D. A., & Tsagarakis, S. (2022) · Current Opinion in Endocrinology Diabetes and Obesity · doi:10.1097/med.0000000000000725 · Cited by 5 · see citing works
Gene Mutations in Cushing’s Syndrome
Ojha, U., Ogunmwonyi, I., Xiang, J., & Ojha, H. (2022) · Molecular Syndromology · doi:10.1159/000524267 · Cited by 5 · see citing works
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- Authors
- Bourdeau, I., Parisien-La Salle, S., & Lacroix, A.
- Affiliations
- Centre Hospitalier de l’Université de Montréal (CA)
- Corresponding author
- Lacroix, André
- Venue
- Best Practice & Research Clinical Endocrinology & Metabolism
- Publisher
- Elsevier BV
- Published
- 2020-05
- Volume
- 34
- Issue
- 3
- Pages
- 101386
- ISSN
- 1521-690X (ISSN-L)
- OpenAlex
- W3004754163
- Cited by
- 48
- References
- 114
- Citations by year
- 2026: 7 · 2025: 2 · 2024: 6 · 2023: 7 · 2022: 17 · 2021: 7 · 2020: 1
- FWCI
- 1.82
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Best Practice & Research Clinical Endocrinology & Metabolism (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 › Hormonal Regulation and Hypertension
- Keywords
- adrenocortical hyperplasia, primary pigmented nodular adrenocortical disease, Cushing's syndrome, Cushing's disease, ectopic ACTH syndrome, congenital adrenal hyperplasia, primary aldosteronism, cortisol excess
- MeSH terms
- Adrenal Cortex Diseases, Adrenal Gland Neoplasms, Adrenal Glands, Adrenal Hyperplasia, Congenital, Cushing Syndrome, Humans, Hydrocortisone, Hyperaldosteronism, Hyperplasia, Metabolism, Inborn Errors, Receptors, Glucocorticoid
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