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

Authors :: Gevers EF
Meredith S
Shah P
Torpiano J
Peters C
Sebire NJ
Slater O
White A
Dattani MT
Source :: European journal of endocrinology [Eur J Endocrinol] 2017 Feb; Vol. 176 (2), pp. K1-K7. Date of Electronic Publication: 2016 Nov 22.
Publication Year :: 2017
Abstract: Context: Pituitary microadenomas and adrenal tumours are the most common causes for endogenous Cushing syndrome (CS) in children.<br />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.<br />Conclusion: We describe a yolk sac tumour as a novel source of ectopic POMC production leading to CS in a young girl.<br /> (© 2017 European Society of Endocrinology.)

Subjects :: Abdominal Neoplasms blood
Abdominal Neoplasms metabolism
Adrenocorticotropic Hormone blood
Child, Preschool
Cushing Syndrome blood
Endodermal Sinus Tumor blood
Endodermal Sinus Tumor metabolism
Female
Humans
Abdominal Neoplasms complications
Cushing Syndrome etiology
Endodermal Sinus Tumor complications
Pro-Opiomelanocortin metabolism

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