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Clinical profile and treatment outcomes of patients with ectopic ACTH syndrome compared to Cushing disease: a single-center experience.
- Source :
-
Endocrine [Endocrine] 2023 May; Vol. 80 (2), pp. 408-418. Date of Electronic Publication: 2023 Jan 07. - Publication Year :
- 2023
-
Abstract
- Purpose: We aimed to evaluate and compare the clinical, biochemical and radiological profile and outcomes of patients with ectopic ACTH syndrome (EAS) and Cushing disease (CD) treated over a period of 10 years (2013-2022).<br />Methods: In this ambispective observational study, we collected data for 146 patients with ACTH-dependent CS (EAS, n = 23; CD, n = 94; occult ACTH source, n = 29). Relevant details were filled in a predesigned proforma and outcomes were ascertained at the most recent visit.<br />Results: EAS was more common in males (65.2 vs. 27.6%, p < 0.001). Patients with EAS had a shorter duration of symptoms [12 (6-12) vs. 31.5 (15-48) months, p < 0.001] and were more likely to have hypokalemia (82.6 vs. 21.0%, p = 0.001), pedal edema (65.2 vs. 34.2%, p = 0.015), weight loss (34.8 vs. 4.0%, p < 0.001) and systemic infection (30.4 vs. 6.5%, p = 0.006). They also had significantly higher 8 a.m. serum cortisol, midnight serum and salivary cortisol and 8 a.m. plasma ACTH levels. Bronchial carcinoid (n = 10, 43.5%) was the most common etiology of EAS. Bilateral adrenalectomy was performed in 11 (47.8%) patients with EAS. Eight patients (34.8%) with EAS died at the last follow-up, of whom 7 (87.5%) had metastatic disease. In CD group, overall remission rate was 69.4% (56.1%, early and 13.3%, delayed) and 26.3% of patients with an initial remission had recurrence.<br />Conclusions: Bronchial carcinoid was the most common cause of EAS in our cohort. Bilateral adrenalectomy was performed in approximately every 1 in 2 patients with EAS and approximately every 1 in 3 patients expired till the last follow-up.<br /> (© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)
- Subjects :
- Male
Humans
Hydrocortisone
Adrenocorticotropic Hormone
Treatment Outcome
ACTH Syndrome, Ectopic etiology
ACTH Syndrome, Ectopic therapy
Pituitary ACTH Hypersecretion therapy
Pituitary ACTH Hypersecretion complications
Cushing Syndrome
Bronchial Neoplasms complications
Bronchial Neoplasms diagnosis
Carcinoid Tumor complications
Carcinoid Tumor therapy
Subjects
Details
- Language :
- English
- ISSN :
- 1559-0100
- Volume :
- 80
- Issue :
- 2
- Database :
- MEDLINE
- Journal :
- Endocrine
- Publication Type :
- Academic Journal
- Accession number :
- 36609908
- Full Text :
- https://doi.org/10.1007/s12020-022-03298-z