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Related Concept Videos

Cushing Syndrome I: Introduction01:26

Cushing Syndrome I: Introduction

Cushing syndrome refers to the collection of clinical manifestations that arise when tissues are exposed to excessive amounts of cortisol or cortisol-like medications over an extended period. Cortisol, a glucocorticoid produced by the adrenal cortex, regulates metabolism, immune responses, and the body’s adaptation to stress. When its concentration remains chronically elevated, these physiological pathways become dysregulated, resulting in the characteristic features of the syndrome.Exogenous...
Cushing Syndrome II: Pathophysiology01:19

Cushing Syndrome II: Pathophysiology

Cortisol production is normally governed by the hypothalamic–pituitary–adrenal (HPA) axis, which maintains hormonal balance through tightly regulated feedback mechanisms. Disruption of this regulatory system is central to the development of Cushing syndrome, whether the excess cortisol originates from external medications or internal pathology. Persistent cortisol elevation alters metabolism, immune function, and endocrine signaling, producing the characteristic clinical features of the...
Adrenal Gland Disorders01:27

Adrenal Gland Disorders

Adrenal gland disorders manifest when the production of adrenal hormones deviates from the norm, resulting in either excessive or insufficient concentrations.
Adrenal insufficiency, characterized by insufficient cortisol and aldosterone production, leads to conditions like Addison's disease. This disorder, affecting the adrenal cortex, exhibits symptoms such as skin bronzing, dehydration, low blood pressure, fatigue, and weight loss. Congenital adrenal hyperplasia, a genetic ailment causing...
Hypothalamic-Pituitary Axis01:37

Hypothalamic-Pituitary Axis

The response to stress—be it physical or psychological, acute or chronic—involves activation of the Hypothalamic-Pituitary-Adrenal (HPA) axis. The HPA axis is part of the neuroendocrine system because it involves both neuronal and hormonal communication. Its function is to regulate homeostatic systems—metabolic, cardiovascular, and immune—providing the necessary means to respond to a stressor.

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Related Experiment Video

Updated: Jul 19, 2026

A Novel Method: Super-selective Adrenal Venous Sampling
06:08

A Novel Method: Super-selective Adrenal Venous Sampling

Published on: September 15, 2017

Ectopic ACTH syndrome: our experience with 25 cases.

Luiz Roberto Salgado1, Maria Candida B Villares Fragoso, Mirta Knoepfelmacher

  • 1Division of Endocrinology and Metabolism, Hospital das Clínicas, University of Sao Paulo Medical School, Avenida Dr Eneas de Carvalho Aguiar, 155, 8th floor, Cerqueira Cesar, 05403-060 Sao Paulo, Brazil. salga@uol.com.br

European Journal of Endocrinology
|October 26, 2006
PubMed
Summary

Ectopic ACTH syndrome (EAS) diagnosis involves dynamic tests and imaging. Inferior petrosal sinus sampling (IPSS) with desmopressin aids differential diagnosis, with thymic carcinoids indicating a poor prognosis.

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Endoscopic Endonasal Trans-sphenoidal Approach: Minimally Invasive Surgery for Pituitary Adenomas
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Endoscopic Endonasal Trans-sphenoidal Approach: Minimally Invasive Surgery for Pituitary Adenomas

Published on: January 17, 2018

Area of Science:

  • Endocrinology
  • Oncology

Background:

  • Ectopic ACTH syndrome (EAS) accounts for 5-10% of ACTH-dependent hypercortisolism, often linked to intrathoracic neoplasms.
  • Tumors causing EAS can be overt malignancies or occult, indolent types.

Purpose of the Study:

  • To evaluate the diagnostic accuracy of dynamic tests, IPSS with desmopressin, and imaging for EAS.
  • To compare prognoses of different tumor types associated with EAS.

Main Methods:

  • Reviewed tumor markers, imaging, and outcomes in 25 EAS patients (aged 18-72).
  • Assessed High Dexamethasone Suppression Test (HDDST), desmopressin, GHRP-6, CRH tests, IPSS, CT, MRI, and (111)In-pentetreotide scintigraphy.

Main Results:

  • HDDST was positive in 5/20 patients. Desmopressin test showed ACTH/cortisol responses in 6/13 and 5/13 patients, respectively.
  • CT identified tumors in 15/21 patients, MRI in 8/17. IPSS with desmopressin showed no ACTH gradients in 6/7 patients.
  • Intrathoracic tumors were most common (14/25), followed by pheochromocytomas (5/25). Metastasis correlated with mortality.

Conclusions:

  • IPSS with desmopressin proved useful for differential diagnosis in EAS.
  • Occult carcinoids can cause severe hypercortisolism; thymic carcinoids have poorer prognoses than bronchial carcinoids or pheochromocytomas.