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Updated: Jun 13, 2026

A Novel Method: Super-selective Adrenal Venous Sampling
Published on: September 15, 2017
Ectopic Cushing's syndrome due to an adrenal ganglioneuroma
Jean-Benoît Corcuff1, Colette Deminiere, Jacqueline Trouillas
1Department of Nuclear Medicine, CHU Bordeaux, Bordeaux, France. jean-benoit.corcuff@chu-bordeaux.fr
Background:
Cushing's syndrome (CS), rare in children, is due to pituitary or, less frequently, to adrenocortical tumors. Ectopic adrenocorticotropin (ACTH) secretion is exceptional.
Method:
A case of apparently ACTH-independent CS in a child is reported.
Results:
CS was due to an adrenal ganglioneuroma where neuroendocrine cells were immunopositive for ACTH responsible for the syndrome through a paracrine effect. Cortical cell hyperplasia was observed.
Conclusion:
Benign and differentiated tumors of the neural crest such as ganglioneuromas may be responsible for CS.
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