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.


METHODS
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.


CONCLUSIONS
Benign and differentiated tumors of the neural crest such as ganglioneuromas may be responsible for CS.
Corcuff
Jean-Benoît
JB
Department of Nuclear Medicine, CHU Bordeaux, Bordeaux, France. jean-benoit.corcuff@chu-bordeaux.fr
Deminiere
Colette
C
Trouillas
Jacqueline
J
Puel
Olivier
O
Perel
Yves
Y
Barat
Pascal
P
D000293:Adolescent; D000306:Adrenal Cortex Neoplasms / Q000175:diagnosis / Q000473:pathology*; D000311:Adrenal Glands / Q000473:pathology; D000324:Adrenocorticotropic Hormone / Q000378:metabolism; D003480:Cushing Syndrome / Q000175:diagnosis / Q000209:etiology* / Q000473:pathology; D005260:Female; D005729:Ganglioneuroma / Q000175:diagnosis / Q000473:pathology*; D006729:Hormones, Ectopic / Q000378:metabolism; D006801:Humans
D006729:Hormones, Ectopic; D000324:Adrenocorticotropic Hormone