An adolescent with food-dependent Cushing's syndrome secondary to ectopic expression of GIP receptor in unilateral adrenal adenoma

J Pediatr Endocrinol Metab. 2002 Jun;15(6):853-60. doi: 10.1515/jpem.2002.15.6.853.

Abstract

ACTH-independent Cushing's syndrome may be secondary to the expression of ectopic hormone receptors in adrenal tissue. In food-dependent Cushing's syndrome the adrenals aberrantly express receptors for gastric inhibitory polypeptide (GIP). We present the first case of food- and GIP-dependent adrenal adenoma in an adolescent. A 15 year-old girl presented with signs of hypercortisolism. Urinary cortisol excretion was clearly elevated. Upon the finding of very low fasting plasma cortisol levels with a rise during the morning, food-dependent cortisol secretion was suspected. Hourly measurements of plasma cortisol and GIP levels during a day with and a day without meals showed meal- and GIP-related cortisol secretion. Plasma ACTH levels were undetectable. Abdominal computed tomography showed a tumor of 2.5 x 2.5 x 2 cm in the left adrenal. Unilateral adrenalectomy was performed and microscopic examination of the tumor showed an adrenocortical adenoma. Incubation of adenomatous cells prepared from this tumor with GIP resulted in increased cortisol secretion. Using RT-PCR amplification and cDNA hybridization, the GIP receptor was found to be overexpressed in the adenoma tissue but not in the adjacent adrenal tissue. Twelve months after the operation, hourly measurements of plasma cortisol, GIP and ACTH levels on a day with and a day without meals no longer showed GIP-related cortisol production. The present report shows that in an adolescent with corticotrophin-independent Cushing's syndrome, food-dependent hypercortisolism is a possible diagnosis.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adenoma / metabolism*
  • Adenoma / pathology
  • Adenoma / surgery
  • Adolescent
  • Adrenal Gland Neoplasms / metabolism*
  • Adrenal Gland Neoplasms / pathology
  • Adrenal Gland Neoplasms / surgery
  • Adrenalectomy
  • Cushing Syndrome / etiology*
  • Cushing Syndrome / genetics
  • Cushing Syndrome / surgery
  • Dexamethasone
  • Eating / physiology*
  • Female
  • Glucocorticoids
  • Humans
  • Hydrocortisone / blood
  • Hydrocortisone / urine
  • In Situ Hybridization
  • Radioimmunoassay
  • Receptors, Gastrointestinal Hormone / genetics
  • Receptors, Gastrointestinal Hormone / metabolism*
  • Reverse Transcriptase Polymerase Chain Reaction

Substances

  • Glucocorticoids
  • Receptors, Gastrointestinal Hormone
  • Dexamethasone
  • gastric inhibitory polypeptide receptor
  • Hydrocortisone