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.
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Authors | Vikrant Gosavi, Anurag Lila, Saba Samad Memon, Vijaya Sarathi, Kunal Thakkar, Abhay Dalvi, Gaurav Malhotra, Gagan Prakash, Virendra Patil, Nalini S Shah, Tushar Bandgar |
Journal | Hormone and metabolic research = Hormon- und Stoffwechselforschung = Hormones et metabolisme
(Horm Metab Res)
Vol. 54
Issue 2
Pg. 57-66
(Feb 2022)
ISSN: 1439-4286 [Electronic] Germany |
PMID | 35130566
(Publication Type: Journal Article, Observational Study)
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Copyright | Thieme. All rights reserved. |
Chemical References |
- Adrenocorticotropic Hormone
- Hydrocortisone
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Topics |
- Adrenal Cortex Neoplasms
(complications)
- Adrenocorticotropic Hormone
- Cushing Syndrome
- Humans
- Hydrocortisone
- Hyperplasia
(complications)
- Retrospective Studies
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