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Published in: Journal of Endocrinological Investigation 3/2024

Open Access 05-10-2023 | Cushing's Syndrome | Original Article

Corticotropin-releasing hormone test predicts the outcome of unilateral adrenalectomy in primary bilateral macronodular adrenal hyperplasia

Authors: I. Tizianel, M. Detomas, T. Deutschbein, M. Fassnacht, N. Albiger, M. Iacobone, C. Scaroni, F. Ceccato

Published in: Journal of Endocrinological Investigation | Issue 3/2024

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Abstract

Purpose

Primary bilateral adrenal hyperplasia (PBMAH) is associated with hypercortisolism and a heterogeneous clinical expression in terms of cortisol secretion and related comorbidities. Historically, treatment of choice was bilateral adrenalectomy (B-Adx); however, recent data suggest that unilateral adrenalectomy (U-Adx) may be an effective alternative. For the latter, factors predicting the postsurgical outcome (e.g., biochemical control) have not been identified yet.

Methods

PBMAH patients undergoing U-Adx for overt Cushing’s syndrome (CS) in two tertiary care centers were retrospectively analysed. Remission was defined as a normalization of urinary free cortisol (UFC) without the need for medical treatment. The potential of hCRH test as a predictor of U-Adx outcome was evaluated in a subgroup.

Results

23 patients were evaluated (69% females, mean age 55 years). Remission rate after U-Adx was 74% at last follow up (median 115 months from UAdx). Before U-Adx, a positive ACTH response to hCRH (Δ%ACTH increase > 50% from baseline) was associated with higher remission rates.

Conclusions

Three of four patients with PBMAH are surgically cured with U-Adx. Pre-operative hCRH testing can be useful to predict long-term remission rates.
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Literature
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go back to reference Stratakis CA (2008) Cushing syndrome caused by adrenocortical tumors and hyperplasias (Corticotropin-independent cushing syndrome). In: Flück CE, Miller WL (eds) Disorders of the human adrenal cortex. KARGER, Basel, pp b117-132CrossRef Stratakis CA (2008) Cushing syndrome caused by adrenocortical tumors and hyperplasias (Corticotropin-independent cushing syndrome). In: Flück CE, Miller WL (eds) Disorders of the human adrenal cortex. KARGER, Basel, pp b117-132CrossRef
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go back to reference 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 (2010) 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. Eur J Endocrinol 163(1):129–138. https://doi.org/10.1530/EJE-10-0195CrossRefPubMed 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 (2010) 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. Eur J Endocrinol 163(1):129–138. https://​doi.​org/​10.​1530/​EJE-10-0195CrossRefPubMed
Metadata
Title
Corticotropin-releasing hormone test predicts the outcome of unilateral adrenalectomy in primary bilateral macronodular adrenal hyperplasia
Authors
I. Tizianel
M. Detomas
T. Deutschbein
M. Fassnacht
N. Albiger
M. Iacobone
C. Scaroni
F. Ceccato
Publication date
05-10-2023
Publisher
Springer International Publishing
Published in
Journal of Endocrinological Investigation / Issue 3/2024
Electronic ISSN: 1720-8386
DOI
https://doi.org/10.1007/s40618-023-02204-2

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