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Updated: May 8, 2026
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A Novel Method: Super-selective Adrenal Venous Sampling
Published on: September 15, 2017
Ectopic ACTH-secreting syndrome: a single-center experience
Harshal R Kakade1, Rajeev Kasaliwal, Varsha S Jagtap
1Department of Endocrinology, Seth G S Medical College, KEM Hospital, Mumbai, India.
Summary
Ectopic adrenocorticotropic hormone (ACTH)-secreting syndrome (EAS) can occur even with normal ACTH levels. Imaging like CT and PET scans are crucial for locating the source, with high cure rates for bronchial carcinoids.
Area of Science:
- Endocrinology
- Oncology
- Radiology
Background:
- Ectopic adrenocorticotropic hormone (ACTH)-secreting syndrome (EAS) is a rare endocrine disorder causing hypercortisolism.
- Accurate diagnosis and localization are challenging due to varied clinical presentations.
Purpose of the Study:
- To analyze the clinical, biochemical, and imaging features of EAS patients.
- To evaluate management strategies and patient outcomes.
- To identify optimal diagnostic and localization techniques for EAS.
Main Methods:
- Retrospective review of 218 patients with ACTH-dependent hypercortisolism (1993-2012).
- Analysis of clinical data, hormone levels (serum cortisol, plasma ACTH), and imaging results (pituitary MRI, CT, Gallium DOTATATE PET).
- Evaluation of treatment outcomes including cure rates.
Main Results:
- 17 patients were diagnosed with EAS, with median serum cortisol of 36 μg/dL and ACTH of 156 pg/mL.
- ACTH levels below 100 pg/mL were observed in 4 patients, highlighting diagnostic challenges.
- Computed tomography (CT) localized the ectopic source in 15/17 patients; Gallium DOTATATE PET was effective when CT failed.
- High cure rate (72%) was observed, though outcomes varied by tumor type (bronchial vs. thymic carcinoids).
Conclusions:
- Ectopic ACTH-secreting syndrome should be considered even with normal or marginally elevated ACTH levels.
- Inferior petrosal sinus sampling (IPSS) can prevent unnecessary pituitary surgery when MRI findings are equivocal.
- CT is valuable for localization, with functional imaging like PET scans useful in difficult cases.
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