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A Novel Method: Super-selective Adrenal Venous Sampling
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Ectopic Cushing syndrome in Colombia.

Vanessa Lopez-Montoya1, Johnayro Gutierrez-Restrepo2, Jose Luis Torres Grajales3

  • 1Facultad de Medicina, Universidad de Antioquia, MedellĂ­n, Colombia.

Archives of Endocrinology and Metabolism
|May 25, 2021
PubMed
Summary
This summary is machine-generated.

Ectopic Cushing syndrome (ECS) is a rare condition with high mortality. Early diagnosis and multidisciplinary intervention are crucial for improving patient outcomes and survival rates.

Keywords:
Cushing syndromecarcinoidsectopic ACTH production syndromeneuroendocrine tumors

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Area of Science:

  • Endocrinology
  • Oncology

Background:

  • Ectopic Cushing syndrome (ECS) is a rare endocrine disorder caused by non-pituitary, non-adrenal tumors secreting adrenocorticotropic hormone (ACTH).
  • Clinical presentation and management of ECS can be challenging due to its rarity and diverse etiologies.

Purpose of the Study:

  • To describe the clinical characteristics of Colombian patients with ectopic Cushing syndrome (ECS).
  • To compare these findings with existing literature to optimize patient management strategies.

Main Methods:

  • Retrospective review of medical records for patients diagnosed with ECS between 1986 and 2017.
  • Exclusion of patients with adrenal or pituitary Cushing syndrome (CS).

Main Results:

  • Fourteen ECS patients were analyzed; common etiologies included lung carcinoids, small-cell lung carcinoma, and pancreatic neuroendocrine tumors.
  • Frequent clinical features were moon-face, muscle weakness, diabetes mellitus, and hypertension, with 36% exhibiting hyperpigmentation and 92% having identified primary lesions.
  • High morbidity and mortality were observed, with a 57.1% death rate and a median overall survival of 27 months; intrathoracic tumors showed aggressive behavior.

Conclusions:

  • Ectopic Cushing syndrome (ECS) is a rare but severe condition associated with significant morbidity and mortality.
  • Prompt diagnosis and intervention by an interdisciplinary team are essential for improving survival and quality of life in ECS patients.