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Published in: BMC Endocrine Disorders 1/2015

Open Access 01-12-2015 | Case report

Paediatric cyclical Cushing’s disease due to corticotroph cell hyperplasia

Authors: E. Noctor, S. Gupta, T. Brown, M. Farrell, M. Javadpour, C. Costigan, A. Agha

Published in: BMC Endocrine Disorders | Issue 1/2015

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Abstract

Background

Cushing’s disease is very rare in the paediatric population. Although uncommon, corticotroph hyperplasia causing Cushing’s syndrome has been described in the adult population, but appears to be extremely rare in children. Likewise, cyclical cortisol hypersecretion, while accounting for 15 % of adult cases of Cushing’s disease, has only rarely been described in the paediatric population. Here, we describe a very rare case of a 13-year old boy with cyclical cortisol hypersecretion secondary to corticotroph cell hyperplasia.

Case presentation

The case is that of a 13-year old boy, presenting with a long history of symptoms and signs suggestive of hypercortisolism, who was found to have cyclical ACTH-dependent hypercortisolism following dynamic pituitary testing and serial late-night salivary cortisol measurements. The patient underwent endoscopic transsphenoidal resection of the pituitary. Early surgical remission was confirmed by undetectable post-operative morning plasma cortisol levels. Histology and immunocytochemistry of the resected pituitary tissue showed extensive corticotroph cell hyperplasia.

Conclusion

This report describes a rare case of cyclical Cushing’s disease secondary to corticotroph hyperplasia in a paediatric patient. This highlights the challenging and varied nature of Cushing’s disease and its diagnosis, and the need to keep a differential diagnosis in mind during the diagnostic process.
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Metadata
Title
Paediatric cyclical Cushing’s disease due to corticotroph cell hyperplasia
Authors
E. Noctor
S. Gupta
T. Brown
M. Farrell
M. Javadpour
C. Costigan
A. Agha
Publication date
01-12-2015
Publisher
BioMed Central
Published in
BMC Endocrine Disorders / Issue 1/2015
Electronic ISSN: 1472-6823
DOI
https://doi.org/10.1186/s12902-015-0024-3

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