title: Adrenocorticotropic hormone independent macronodular adrenal hyperplasia due to aberrant receptor expression: is medical treatment always an option?
abstract: OBJECTIVE
  To investigate the efficacy of medical treatment as an alternative option to bilateral adrenalectomy in patients with cortisol excess due to adrenocorticotropic hormone (ACTH) independent macronodular adrenal hyperplasia (AIMAH).
  
  
  METHODS
  We focused on the efficacy of somatostatin analogues in a patient with food-dependent AIMAH and of leuprolide acetate in a patient with AIMAH due to aberrant LH/hCG receptor expression.
  
  
  RESULTS
  Two female patients with bilateral macronodular adrenal hyperplasia and cortisol excess were evaluated for the presence of aberrant cortisol responses. One patient demonstrated an aberrant response to mixed meal and the other, a menopausal female, to luteinizing hormone-releasing hormone (LHRH) and human chorionic gonadotropin (hCG) administration. In the first patient, subcutaneous octreotide was administered prior to mixed meal and completely abolished food-induced cortisol secretion. Thus, the patient was treated with the long-acting somatostatin analogue octreotide long-acting release (LAR) for 3 months. There was no control of cortisol excess upon reevaluation and acute subcutaneous octreotide administration prior to meal was no longer effective in blocking food-induced cortisol secretion. The second patient successfully responded to leuprolide acetate and, for 40 months, her cortisol excess remains in long-term control.
  
  
  CONCLUSIONS
  A luteinizing hormone/human chorionic gonadotropin (LH/hCG) responsive patient with AIMAH sustained long-term control of cortisol excess on leuprolide acetate. In contrast, in a meal-responsive patient with apparent gastric inhibitory polypeptide (GIP) dependent AIMAH, did not achieve remission under somatostatin analogues.
authors: Department of Endocrinology, Diabetes and Metabolism, Evangelismos Hospital, Athens, Greece.
authors: Olga
authors: O
authors: Karapanou
authors: Barbara
authors: B
authors: Vlassopoulou
authors: Marinella
authors: M
authors: Tzanela
authors: Theodora
authors: T
authors: Stratigou
authors: Vassilios
authors: V
authors: Tsatlidis
authors: Sofia
authors: S
authors: Tsirona
authors: Stylianos
authors: S
authors: Tsagarakis
mesh_terms: D000328:Adult; D006063:Chorionic Gonadotropin / Q000627:therapeutic use; D003480:Cushing Syndrome / Q000188:drug therapy / Q000378:metabolism*; D005260:Female; D006801:Humans; D007986:Luteinizing Hormone / Q000627:therapeutic use; D008875:Middle Aged; D015282:Octreotide / Q000627:therapeutic use
chemical_list: D006063:Chorionic Gonadotropin; D007986:Luteinizing Hormone; D015282:Octreotide