title: Infrasellar pituitary gangliocytoma causing Cushing's syndrome.
abstract: BACKGROUND
  Pituitary gangliocytomas are uncommon neuronal tumours that may present with endocrine disorders, the most frequent being acromegaly caused by growth hormone hypersecretion. Cushing's syndrome is very rarely seen with gangliocytomas.
  
  
  METHODS
  We report the unique case of a 62 year-old woman whose clinical picture and endocrine testing clearly demonstrated adrenocorticotropin (ACTH)-dependent Cushing's syndrome. Pituitary magnetic resonance imaging showed a 12-mm homogeneous, infra- and retrosellar mass first diagnosed as pituitary macroadenoma. Transsphenoidal surgery was performed and allowed complete resection of the tumour with sparing of normal anterior pituitary. Very low postoperative serum cortisol and ACTH levels were observed in the early postoperative period and the patient is still in remission 18 months after surgery, thus demonstrating that the resected lesion was entirely responsible for the clinical picture.
  
  
  RESULTS
  Histological and immunocytochemical analyses demonstrated a benign tumour composed of mature neuronal cells suggestive of a gangliocytoma, expressing both ACTH and corticotropin-releasing hormone (CRH). The tumour was surrounded by a rim of pituitary tissue containing ACTH-producing endocrine cells. Careful analysis of the resected lesion did not reveal any pituitary microadenoma. We search literature for similar cases and retraced only nine cases of gangliocytomas associated with Cushing's syndrome. In most of them, the tumour was combined with either pituitary corticotroph adenoma or hyperplasia.
  
  
  CONCLUSIONS
  Our case represents a unique case of an infrasellar pituitary gangliocytoma which was able to cause Cushing's syndrome by both direct ACTH production and CRH-induced stimulation of neighbour normal corticotroph cells.
authors.affiliation: Department of Endocrinology and Nutrition, Cliniques Universitaires St-Luc, Avenue Hippocrate 10, 54.74, 1200, Brussels, Belgium.
authors.forename: Marie-Eve
authors.initials: ME
authors.lastname: Domingue
authors.forename: Etienne
authors.initials: E
authors.lastname: Marbaix
authors.forename: Jean-Luc
authors.initials: JL
authors.lastname: Do Rego
authors.forename: Vincent
authors.initials: V
authors.lastname: Col
authors.forename: Christian
authors.initials: C
authors.lastname: Raftopoulos
authors.forename: Thierry
authors.initials: T
authors.lastname: Duprez
authors.forename: Hubert
authors.initials: H
authors.lastname: Vaudry
authors.forename: Dominique
authors.initials: D
authors.lastname: Maiter
mesh_terms: D000324:Adrenocorticotropic Hormone / Q000097:blood / Q000378:metabolism; D001706:Biopsy; D003346:Corticotropin-Releasing Hormone / Q000097:blood / Q000378:metabolism; D003480:Cushing Syndrome / Q000097:blood / Q000175:diagnosis / Q000209:etiology*; D005260:Female; D005729:Ganglioneuroma / Q000097:blood / Q000150:complications* / Q000175:diagnosis / Q000378:metabolism / Q000601:surgery; D006801:Humans; D007016:Hypophysectomy; D007150:Immunohistochemistry; D008279:Magnetic Resonance Imaging; D008875:Middle Aged; D010911:Pituitary Neoplasms / Q000097:blood / Q000150:complications* / Q000175:diagnosis / Q000378:metabolism / Q000601:surgery; D016896:Treatment Outcome; D047368:Tumor Burden
chemical_list: D000324:Adrenocorticotropic Hormone; D003346:Corticotropin-Releasing Hormone