Treatment of adrenocorticotropin-dependent Cushing's syndrome: a consensus statement.
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.
Biller
B M K
BM
Institute of Internal Medicine, Division of Endocrinology, School of Medicine, Polytechnic University of Marche, Torrette, Ancona, Italy.
Grossman
A B
AB
Stewart
P M
PM
Melmed
S
S
Bertagna
X
X
Bertherat
J
J
Buchfelder
M
M
Colao
A
A
Hermus
A R
AR
Hofland
L J
LJ
Klibanski
A
A
Lacroix
A
A
Lindsay
J R
JR
Newell-Price
J
J
Nieman
L K
LK
Petersenn
S
S
Sonino
N
N
Stalla
G K
GK
Swearingen
B
B
Vance
M L
ML
Wass
J A H
JA
Boscaro
M
M
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
D008939:Mitotane; D000324:Adrenocorticotropic Hormone; D008797:Metyrapone