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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