Ectopic Cushing's syndrome due to an adrenal ganglioneuroma

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


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.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Adrenal Cortex Neoplasms / diagnosis
  • Adrenal Cortex Neoplasms / pathology*
  • Adrenal Glands / pathology
  • Adrenocorticotropic Hormone / metabolism
  • Cushing Syndrome / diagnosis
  • Cushing Syndrome / etiology*
  • Cushing Syndrome / pathology
  • Female
  • Ganglioneuroma / diagnosis
  • Ganglioneuroma / pathology*
  • Hormones, Ectopic / metabolism
  • Humans


  • Hormones, Ectopic
  • Adrenocorticotropic Hormone