Ectopic cushing’s syndrome due to an adrenal ganglioneuroma

Jean-Benoît Corcuff, Colette Deminiere, Jacqueline Trouillas, Olivier Puel, Yves Perel, Pascal Barat
Horm Res Paediatr. 2010-01-01; 73(5): 405-408
DOI: 10.1159/000308175

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1. Horm Res Paediatr. 2010;73(5):405-8. doi: 10.1159/000308175. Epub 2010 Apr 14.

Ectopic Cushing’s syndrome due to an adrenal ganglioneuroma.

Corcuff JB(1), Deminiere C, Trouillas J, Puel O, Perel Y, Barat P.

Author information:
(1)Department of Nuclear Medicine, CHU Bordeaux, Bordeaux, France.

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.

Copyright 2010 S. Karger AG, Basel.

DOI: 10.1159/000308175
PMID: 20389113 [Indexed for MEDLINE]

Auteurs Bordeaux Neurocampus