Second-Line Tests in the Diagnosis of Adrenocorticotropic Hormone-Dependent Hypercortisolism

Cushing's syndrome (CS) is a rare disease caused by chronic and excessive cortisol secretion. When adrenocorticotropin hormone (ACTH) is measurable, autonomous adrenal cortisol secretion could be reasonably ruled out in a differential diagnosis of CS. ACTH-dependent CS accounts for 80%-85% of cases and involves cortisol production stimulated by uncontrolled pituitary or ectopic ACTH secretion. Pituitary adenoma is not detected in up to one-third of cases with pituitary ACTH secretion, whereas cases of CS due to ectopic ACTH secretion may be associated with either malignant neoplasia (such as small cell lung carcinoma) or less aggressive neuroendocrine tumors, exhibiting only the typical symptoms and signs of CS. Since the differential diagnosis of ACTH-dependent CS may be a challenge, many strategies have been proposed. Since none of the available tests show 100% diagnostic accuracy, a step-by-step approach combining several diagnostic tools and a multidisciplinary evaluation in a referral center is suggested. In this review, we present a clinical case to demonstrate the diagnostic work-up of ACTH-dependent CS. We describe the most commonly used dynamic tests, as well as the applications of conventional or nuclear imaging and invasive procedures.

Medienart:

E-Artikel

Erscheinungsjahr:

2021

Erschienen:

2021

Enthalten in:

Zur Gesamtaufnahme - volume:41

Enthalten in:

Annals of laboratory medicine - 41(2021), 6 vom: 01. Nov., Seite 521-531

Sprache:

Englisch

Beteiligte Personen:

Pinelli, Silvia [VerfasserIn]
Barbot, Mattia [VerfasserIn]
Scaroni, Carla [VerfasserIn]
Ceccato, Filippo [VerfasserIn]

Links:

Volltext

Themen:

9002-60-2
Adrenocorticotropic Hormone
Cushing’s syndrome
Differential diagnosis
Ectopic ACTH secretion
Hydrocortisone
Journal Article
Pituitary adenoma
Review
WI4X0X7BPJ

Anmerkungen:

Date Completed 17.09.2021

Date Revised 02.11.2021

published: Print

Citation Status MEDLINE

doi:

10.3343/alm.2021.41.6.521

funding:

Förderinstitution / Projekttitel:

PPN (Katalog-ID):

NLM326563148