Cushing syndrome in a child due to pro-opiomelanocortin (POMC) secretion from a yolk sac tumor-Reference-Cited by-同舟云学术

Cushing syndrome in a child due to pro-opiomelanocortin (POMC) secretion from a yolk sac tumor

Published:2017-02 Issue:2 Volume:176 Page:K1-K7
ISSN:0804-4643
Container-title:European Journal of Endocrinology
language:
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Author:

Gevers Evelien F¹,Meredith Suzanne²,Shah Pratik¹³,Torpiano John⁴,Peters Catherine¹,Sebire Neil J⁵,Slater Olga⁶,White Anne²,Dattani Mehul T¹³

Affiliation:

1. 1Department of EndocrinologyGreat Ormond Street Hospital for Children, London, UK

2. 2Division of DiabetesEndocrinology and Gastroenterology, Faculty of Biology, Medicine and Health, University of Manchester, Manchester Academic Health Sciences Centre, Manchester, UK

3. 6Section of Genetics and Epigenetics in Health and DiseaseGenetics and Genomic Medicine Programme, UCL Institute of Child Health, London, UK

4. 3Department of PaediatricsPaediatric Endocrine Service, Mater Dei Hospital, Msida, Malta

5. 4Department of HistopathologyGreat Ormond Street Hospital and Institute for Child Health (UCL), London, UK

6. 5Department of OncologyGreat Ormond Street Hospital for Children, London, UK

Abstract

Context Pituitary microadenomas and adrenal tumours are the most common causes for endogenous Cushing syndrome (CS) in children. Case description We describe a two-year old girl with Cushing syndrome due to ectopic pro-opiomelanocortin (POMC) production from an abdominal yolk sac tumor. Cortisol concentrations were elevated but adrenocorticotropic hormone (ACTH) concentrations were equivocal. The use of antibodies specifically detecting ACTH precursors revealed that plasma ACTH precursors were elevated. Additionally, an ACTH assay with a low cross-reactivity for precursors showed low concentrations of ACTH. Immunohistochemistry suggested POMC but not ACTH production by the tumour. Conclusion We describe a yolk sac tumour as a novel source of ectopic POMC production leading to CS in a young girl.

Publisher

Bioscientifica

Subject

Endocrinology,General Medicine,Endocrinology, Diabetes and Metabolism

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