open access

Vol 67, No 4 (2016)
Postgraduate education
Published online: 2016-07-06
Submitted: 2016-01-28
Accepted: 2016-03-22
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Cushing’s syndrome due to ectopic ACTH secretion

Łukasz Cieszyński, Monika Berendt-Obołończyk, Michał Szulc, Krzysztof Sworczak
DOI: 10.5603/EP.a2016.0055
·
Endokrynologia Polska 2016;67(4):458-471.

open access

Vol 67, No 4 (2016)
Postgraduate education
Published online: 2016-07-06
Submitted: 2016-01-28
Accepted: 2016-03-22

Abstract

Cushing’s syndrome (CS) is defined as a constellation of clinical signs and symptoms occurring due to hypercortisolism. Cortisol excess may be endogenous or exogenous. The most common cause of CS is glucocorticoid therapy with supraphysiological (higher than in the case of substitution) doses used in various diseases (e.g. autoimmune). One possible CS cause is ectopic (extra-pituitary) ACTH secretion (EAS) by benign or malignant tumours. Since its first description in 1963, EAS aetiology has changed, i.e. as well as small cell lung cancer (SCLC), higher incidence in other malignancies has been reported. Ectopic ACTH secretion symptoms are usually similar to hypercortisolism symptoms due to other causes. A clinical suspicion of CS requires laboratory investigations. There is no single and specific laboratory test for making a CS diagnosis, and therefore multiple dynamic tests should be ordered. A combination of multiple laboratory noninvasive and invasive tests gives 100% sensitivity and 98% specificity for EAS diagnosis. If the EAS is caused by localised malignancy, surgery is the optimal treatment choice. Radical tumour excision may be performed in 40% of patients, and 80% of them are cured of the disease. The authors present an interesting clinical case of EAS, which is always a huge diagnostic challenge for clinicians. (Endokrynol Pol 2016; 67 (4): 458–464)

Abstract

Cushing’s syndrome (CS) is defined as a constellation of clinical signs and symptoms occurring due to hypercortisolism. Cortisol excess may be endogenous or exogenous. The most common cause of CS is glucocorticoid therapy with supraphysiological (higher than in the case of substitution) doses used in various diseases (e.g. autoimmune). One possible CS cause is ectopic (extra-pituitary) ACTH secretion (EAS) by benign or malignant tumours. Since its first description in 1963, EAS aetiology has changed, i.e. as well as small cell lung cancer (SCLC), higher incidence in other malignancies has been reported. Ectopic ACTH secretion symptoms are usually similar to hypercortisolism symptoms due to other causes. A clinical suspicion of CS requires laboratory investigations. There is no single and specific laboratory test for making a CS diagnosis, and therefore multiple dynamic tests should be ordered. A combination of multiple laboratory noninvasive and invasive tests gives 100% sensitivity and 98% specificity for EAS diagnosis. If the EAS is caused by localised malignancy, surgery is the optimal treatment choice. Radical tumour excision may be performed in 40% of patients, and 80% of them are cured of the disease. The authors present an interesting clinical case of EAS, which is always a huge diagnostic challenge for clinicians. (Endokrynol Pol 2016; 67 (4): 458–464)

Get Citation

Keywords

Cushing`s syndrom; ectopic ACTH secretion; hypercortisolism; neuroendocrine neoplasm

About this article
Title

Cushing’s syndrome due to ectopic ACTH secretion

Journal

Endokrynologia Polska

Issue

Vol 67, No 4 (2016)

Pages

458-471

Published online

2016-07-06

DOI

10.5603/EP.a2016.0055

Bibliographic record

Endokrynologia Polska 2016;67(4):458-471.

Keywords

Cushing`s syndrom
ectopic ACTH secretion
hypercortisolism
neuroendocrine neoplasm

Authors

Łukasz Cieszyński
Monika Berendt-Obołończyk
Michał Szulc
Krzysztof Sworczak

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