Ectopic Cushing's syndrome due to an adrenal ganglioneuroma.

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.