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Pituitary
Erschienen in: Pituitary 5/2018

15.05.2018

Two types of ectopic Cushing syndrome or a continuum? Review

verfasst von: Marta Araujo Castro, Mónica Marazuela Azpiroz

Erschienen in: Pituitary | Ausgabe 5/2018

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Abstract

Background

Two types of ectopic Cushing syndrome (ECS) are described: ECS associated with aggressive neoplasms, and ECS with indolent and occult tumors, however, there is a lack of studies that thoroughly review their characteristics.

Methods

A systematic review was carried out on PUBMED of all the papers about the ECS, in order to better define the types of this subcategory of Cushing’s syndrome, highlighting the differential aspects between these subgroups.

Results

It was found that in 50% of cases the prototypic “aggressive” ECS is caused by small cell lung carcinomas (SCLC). In these cases, the clinical presentation may be atypical, predominating the signs and symptoms derived from the protein catabolism. Cortisol and ACTH levels are extremely high, the clinical presentation is abrupt (< 3–6 months) and the tumor is usually advanced, being impossible a curative treatment. On the other hand, “indolent” ECS is mainly represented by carcinoid tumors (CT). In these cases the clinical presentation overlaps enormously with that of Cushing’s disease (CD). Cortisol and ACTH levels are slightly elevated, the clinical presentation is progressive (> 6 months) and the prognosis is usually good, and a curative treatment is possible in about 75% of the cases.

Conclusion

Although there is no absolute differentiation between the two extremes of ECS, a classification could be established in two groups, guided by its clinical and biochemical characteristics, and mainly by the type and stage of the ACTH-secreting tumor. However, a small percentage of tumors do not fit in this simple grouping, and may present both phenotypes or an intermediate one.
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Metadaten
Titel
Two types of ectopic Cushing syndrome or a continuum? Review
verfasst von
Marta Araujo Castro
Mónica Marazuela Azpiroz
Publikationsdatum
15.05.2018
Verlag
Springer US
Erschienen in
Pituitary / Ausgabe 5/2018
Print ISSN: 1386-341X
Elektronische ISSN: 1573-7403
DOI
https://doi.org/10.1007/s11102-018-0894-2

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