title: Treatment of adrenocorticotropin-dependent Cushing's syndrome: a consensus statement.
abstract: OBJECTIVE
  Our objective was to evaluate the published literature and reach a consensus on the treatment of patients with ACTH-dependent Cushing's syndrome, because there is no recent consensus on the management of this rare disorder.
  
  
  METHODS
  Thirty-two leading endocrinologists, clinicians, and neurosurgeons with specific expertise in the management of ACTH-dependent Cushing's syndrome representing nine countries were chosen to address 1) criteria for cure and remission of this disorder, 2) surgical treatment of Cushing's disease, 3) therapeutic options in the event of persistent disease after transsphenoidal surgery, 4) medical therapy of Cushing's disease, and 5) management of ectopic ACTH syndrome, Nelson's syndrome, and special patient populations.
  
  
  METHODS
  Participants presented published scientific data, which formed the basis of the recommendations. Opinion shared by a majority of experts was used where strong evidence was lacking.
  
  
  METHODS
  Participants met for 2 d, during which there were four chaired sessions of presentations, followed by general discussion where a consensus was reached. The consensus statement was prepared by a steering committee and was then reviewed by all authors, with suggestions incorporated if agreed upon by the majority.
  
  
  CONCLUSIONS
  ACTH-dependent Cushing's syndrome is a heterogeneous disorder requiring a multidisciplinary and individualized approach to patient management. Generally, the treatment of choice for ACTH-dependent Cushing's syndrome is curative surgery with selective pituitary or ectopic corticotroph tumor resection. Second-line treatments include more radical surgery, radiation therapy (for Cushing's disease), medical therapy, and bilateral adrenalectomy. Because of the significant morbidity of Cushing's syndrome, early diagnosis and prompt therapy are warranted.
authors.affiliation: Institute of Internal Medicine, Division of Endocrinology, School of Medicine, Polytechnic University of Marche, Torrette, Ancona, Italy.
authors.forename: B M K
authors.initials: BM
authors.lastname: Biller
authors.forename: A B
authors.initials: AB
authors.lastname: Grossman
authors.forename: P M
authors.initials: PM
authors.lastname: Stewart
authors.forename: S
authors.initials: S
authors.lastname: Melmed
authors.forename: X
authors.initials: X
authors.lastname: Bertagna
authors.forename: J
authors.initials: J
authors.lastname: Bertherat
authors.forename: M
authors.initials: M
authors.lastname: Buchfelder
authors.forename: A
authors.initials: A
authors.lastname: Colao
authors.forename: A R
authors.initials: AR
authors.lastname: Hermus
authors.forename: L J
authors.initials: LJ
authors.lastname: Hofland
authors.forename: A
authors.initials: A
authors.lastname: Klibanski
authors.forename: A
authors.initials: A
authors.lastname: Lacroix
authors.forename: J R
authors.initials: JR
authors.lastname: Lindsay
authors.forename: J
authors.initials: J
authors.lastname: Newell-Price
authors.forename: L K
authors.initials: LK
authors.lastname: Nieman
authors.forename: S
authors.initials: S
authors.lastname: Petersenn
authors.forename: N
authors.initials: N
authors.lastname: Sonino
authors.forename: G K
authors.initials: GK
authors.lastname: Stalla
authors.forename: B
authors.initials: B
authors.lastname: Swearingen
authors.forename: M L
authors.initials: ML
authors.lastname: Vance
authors.forename: J A H
authors.initials: JA
authors.lastname: Wass
authors.forename: M
authors.initials: M
authors.lastname: Boscaro
mesh_terms: D000182:ACTH Syndrome, Ectopic / Q000628:therapy; D000309:Adrenal Insufficiency / Q000628:therapy; D000315:Adrenalectomy; D000324:Adrenocorticotropic Hormone / Q000378:metabolism*; D003480:Cushing Syndrome / Q000628:therapy*; D006801:Humans; D007016:Hypophysectomy; D008797:Metyrapone / Q000627:therapeutic use; D008939:Mitotane / Q000627:therapeutic use; D009347:Nelson Syndrome / Q000628:therapy
chemical_list: D008939:Mitotane; D000324:Adrenocorticotropic Hormone; D008797:Metyrapone