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Related Experiment Videos

Ectopic ACTH syndrome.

Andrea M Isidori1, Andrea Lenzi

  • 1Department of Pathophysiology, Sapienza University of Rome, Italy. andrea.isidori@uniroma1.it

Arquivos Brasileiros De Endocrinologia E Metabologia
|January 23, 2008
PubMed
Summary
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Ectopic adrenocorticotropic secretion (EAS) causes Cushing's syndrome (CS) in 12-17% of cases. This review details diagnosis, localization, and management of EAS, including

Area of Science:

  • Endocrinology
  • Oncology

Background:

  • Ectopic adrenocorticotropic secretion (EAS) accounts for 12-17% of Cushing's syndrome (CS) cases.
  • Severe hypercortisolaemia associated with EAS can worsen the patient's overall condition.
  • EAS diagnosis requires confirming endogenous CS, ACTH dependency, source localization, and biochemical control.

Purpose of the Study:

  • To review trends in prevalence and management of EAS.
  • To discuss diagnostic workup, including dynamic endocrine tests and imaging.
  • To present common causes, localization modalities, and management strategies for EAS.

Main Methods:

  • Systematic review of the largest published series of patients with EAS (over 380 patients).
  • Analysis of diagnostic workup including inferior petrosal sinus sampling with CRH stimulation.

Related Experiment Videos

  • Evaluation of localization studies using high-resolution cross-sectional imaging.

    • Main Results:

    • Revisiting the concept of 'occult' EAS, introducing 'overt' and 'covert' EAS.
    • Common causes include small cell lung carcinoma, bronchial carcinoids, thymic tumours, islet cell tumours, medullary thyroid carcinomas, and phaeochromocytomas.
    • Prevalence and optimal localization modalities for these tumors are presented.

    Conclusions:

    • EAS necessitates a comprehensive diagnostic and management approach.
    • Understanding tumor prevalence and localization is crucial for effective treatment.
    • Medical and surgical management strategies are informed by extensive referral center experience.