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Horm Metab Res 2022; 54(02): 57-66
DOI: 10.1055/a-1735-3232
Endocrine Care

Clinical Spectrum of Adrenal Cushing’s Syndrome and the Caution for Interpretation of Adrenocorticotrophic Hormone: A Single-Center Experience

Vikrant Gosavi 
1   Department of Endocrinology, Seth G. S. Medical College and KEM Hospital, Mumbai, India
,
Anurag Lila
1   Department of Endocrinology, Seth G. S. Medical College and KEM Hospital, Mumbai, India
,
Saba Samad Memon
1   Department of Endocrinology, Seth G. S. Medical College and KEM Hospital, Mumbai, India
,
Vijaya Sarathi
2   Department of Endocrinology, Vydehi Institute of Medical Sciences and Research Centre, Bangalore, India
,
Kunal Thakkar
3   Sterling Ramkrishna Speciality Hospital, Gandhidham, India
,
Abhay Dalvi
4   Department of Surgery, Seth G. S. Medical College and KEM Hospital, Mumbai, India
,
Gaurav Malhotra
5   Department of Nuclear Medicine, Bhabha Atomic Research Centre, Mumbai, India
,
Gagan Prakash
6   Department of Uro-oncology, Tata Memorial Hospital, Mumbai, India
,
Virendra Patil
1   Department of Endocrinology, Seth G. S. Medical College and KEM Hospital, Mumbai, India
,
Nalini S. Shah
1   Department of Endocrinology, Seth G. S. Medical College and KEM Hospital, Mumbai, India
,
Tushar Bandgar
1   Department of Endocrinology, Seth G. S. Medical College and KEM Hospital, Mumbai, India
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Abstract

To describe the differences in presentation, biochemistry, and radiological evaluation of various etiologies of adrenal Cushing’s syndrome (CS) from a single center. To emphasize caution for interpretation of plasma adrenocorticotropic hormone (ACTH), as a spuriously unsuppressed ACTH level by immunometric assay may lead to therapeutic misadventures in adrenal CS. Design: Retrospective, single-center, observational study. Methods:  Fifty-eight adrenal CS patients [Adrenocortical carcinoma (ACC), n=30; Adenoma (ACA), n=15; Primary pigmented nodular adrenocortical disease (PPNAD), n=10; ACTH independent macronodular adrenal hyperplasia (AIMAH), n=3) evaluated at a tertiary care center in western India between January 2006 to March 2020 were included. Data on demography, clinical evaluation, biochemistry, imaging, management, histopathology, and outcome were recorded in a standard format and analyzed. Results:  Cortisol secreting ACC presented at 38(1–50) years with abdominal mass in 26/30 (86.7%) and 16/30 (53.3%) had metastases at presentation. ACA with autonomous cortisol excess presented at 25(4.9–40) years with discriminating features of CS in 14/15 (93.3%), sex steroid production in 2/15, unenhanced HU <10 in only one, and relative washout >40% in 8/11 (72.7%). One ACA and eight ACC patients had plasma ACTH (by Siemens Immulite assay) > 20 pg/ml, despite hypercortisolemic state. Conclusions:  Cortisol-secreting ACC and ACA most often present with mass effects and florid CS, respectively. Baseline HU has low sensitivity to differentiate cortisol-secreting ACA from ACC. Plasma ACTH measured by Seimens Immulite is often unsuppressed, especially in ACC patients, which can be addressed by measuring ACTH by more accurate assays.

Key words

adrenal Cushing’s syndrome - adrenocorticotrophic hormone - Cushing's syndrome - adrenocortical carcinoma


Publication History

Received: 31 August 2021

Accepted after revision: 03 January 2022

Article published online:
07 February 2022

© 2022. Thieme. All rights reserved.

Georg Thieme Verlag
Rüdigerstraße 14, 70469 Stuttgart, Germany

 

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