help button home button Endocrine Society JCEM
HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH
This version published online on April 15, 2008
Journal of Clinical Endocrinology & Metabolism , doi:10.1210/jc.2007-2734
This Article
Right arrow Author Manuscript (PDF)
Right arrow All Versions of this Article:
93/7/2454    most recent
Author Manuscript (PDF)
Right arrow Submit a related Letter to the Editor
Right arrow Alert me when this article is cited
Right arrow Alert me when eLetters are posted
Right arrow Alert me if a correction is posted
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Download to citation manager
Right arrow Reprints, Permissions and Rights
Citing Articles
Right arrow Citing Articles via HighWire
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Biller, B. M.K.
Right arrow Articles by Boscaro, M.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Biller, B. M.K.
Right arrow Articles by Boscaro, M.

Submitted on December 12, 2007
Accepted on April 9, 2008

Treatment of ACTH-dependent Cushing's syndrome: a consensus statement

B. M.K. Biller, A. B. Grossman, P. M. Stewart, S. Melmed, X. Bertagna, J. Bertherat, M. Buchfelder, A. Colao, A. R. Hermus, L. J. Hofland, A. Klibanski, A. Lacroix, J. R. Lindsay, J. Newell-Price, L. K. Nieman, S. Petersenn, N. Sonino, G. K. Stalla, B. Swearingen, M. L. Vance, J. A.H. Wass, and M. Boscaro*

Neuroendocrine Clinical Center, Massachusetts General Hospital (B.M.K.B., A.K., B.S.), Boston, USA; Department of Endocrinology (A.B.G.), St Bartholomew's Hospital, London, UK; Division of Medical Sciences (P.M.S.), University of Birmingham, Queen Elizabeth Hospital, Birmingham, UK; Department of Medicine (S.M.), Cedars Sinai Medical Center, Los Angeles, California, USA; Endocrinology Department (X.B., J.B.), Hôpital Cochin, AP-HP, Faculté de Médecine Paris Descartes, Paris, France; Neurochirurgische Klinik (M.B.), Universität Erlangen, Nürnberg, Germany; Dipartimento di Endocrinologia ed Oncologia Molecolare e Clinica (A.C.), Università degli Studi di Napoli Federico II, Napoli, Italy; Department of Endocrinology (A.R.H.), University Medical Center Nijmegen, The Netherlands; Department of Internal Medicine (L.J.H.), Division of Endocrinology, Erasmus Medical Center, Rotterdam, The Netherlands; Division of Endocrinology (A.L.), Department of Medicine, Centre Hospitalier de l'Université de Montréal, Montréal, Quebec, Canada; Altnagelvin Area Hospital (J.R.L), Londonderry, UK; The Medical School (J.N-P.), University of Sheffield, Sheffield, UK; Reproductive Biology and Medicine Branch (L.K.N.), National Institutes of Health, Bethesda, Maryland, USA; Division of Endocrinology (S.P.), University of Essen, Essen, Germany; Department of Statistical Sciences (N.S.), University of Padova, Padova, Italy; Department of Endocrinology (G.K.S.), Max Planck Institute of Psychiatry, Munich, Germany; University of Virginia Health System (M.L.V.), Charlottesville, Virginia, USA; Department of Endocrinology (J.A.H.W.), Oxford Centre for Diabetes, Endocrinology and Metabolism, Churchill Hospital, Oxford, UK; Division of Endocrinology (M.B), Institute of Internal Medicine, Polytechnic University of Marche, Ancona, Italy

* To whom correspondence should be addressed. E-mail: m.boscaro{at}univpm.it.

Objective: To evaluate the published literature and reach a consensus on the treatment of patients with ACTH-dependent Cushing's syndrome, as there is no recent consensus on the management of this rare disorder.

Participants: 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.

Evidence: 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.

Consensus Process: Participants met for two days, 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 is warranted.
Key words: Cushing's syndrome • Cushing's disease • ACTH • consensus • treatment




This article has been cited by other articles:


Home page
 J. Clin. Endocrinol. Metab.Home page
S. M. Webb, D. Mo, S. W. J. Lamberts, S. Melmed, F. Cavagnini, F. Pecori Giraldi, C. J. Strasburger, A. G. Zimmermann, W. W. Woodmansee, and on behalf of the International HypoCCS Advisory Bo
Metabolic, Cardiovascular, and Cerebrovascular Outcomes in Growth Hormone-Deficient Subjects with Previous Cushing's Disease or Non-Functioning Pituitary Adenoma
J. Clin. Endocrinol. Metab., February 1, 2010; 95(2): 630 - 638.
[Abstract] [Full Text] [PDF]

Home page
 J. Clin. Endocrinol. Metab.Home page
B. Donadille, L. Groussin, C. Waintrop, H. Abbas, F. Tenenbaum, M.-A. Dugue, J. Coste, X. Bertagna, and J. Bertherat
Management of Cushing's Syndrome due to Ectopic Adrenocorticotropin Secretion with 1,Ortho-1, Para'-Dichloro-Diphenyl-Dichloro-Ethane: Findings in 23 Patients from a Single Center
J. Clin. Endocrinol. Metab., February 1, 2010; 95(2): 537 - 544.
[Abstract] [Full Text] [PDF]

Home page
 J. Clin. Endocrinol. Metab.Home page
E. Valassi, B. M. K. Biller, B. Swearingen, F. Pecori Giraldi, M. Losa, P. Mortini, D. Hayden, F. Cavagnini, and A. Klibanski
Delayed Remission after Transsphenoidal Surgery in Patients with Cushing's Disease
J. Clin. Endocrinol. Metab., February 1, 2010; 95(2): 601 - 610.
[Abstract] [Full Text] [PDF]

Home page
 J. Clin. Endocrinol. Metab.Home page
A. Ben-Shlomo, H. Schmid, K. Wawrowsky, O. Pichurin, E. Hubina, V. Chesnokova, N.-A. Liu, M. Culler, and S. Melmed
Differential Ligand-Mediated Pituitary Somatostatin Receptor Subtype Signaling: Implications for Corticotroph Tumor Therapy
J. Clin. Endocrinol. Metab., November 1, 2009; 94(11): 4342 - 4350.
[Abstract] [Full Text] [PDF]

Home page
 radtechHome page
M. E BERRY
Adrenal Gland Disorders
Radiol. Technol., September 1, 2009; 81(1): 57 - 73.
[Abstract] [Full Text] [PDF]

Home page
 J. Clin. Endocrinol. Metab.Home page
F. Castinetti, M. Nagai, I. Morange, H. Dufour, P. Caron, P. Chanson, C. Cortet-Rudelli, J.-M. Kuhn, B. Conte-Devolx, J. Regis, et al.
Long-Term Results of Stereotactic Radiosurgery in Secretory Pituitary Adenomas
J. Clin. Endocrinol. Metab., September 1, 2009; 94(9): 3400 - 3407.
[Abstract] [Full Text] [PDF]

Home page
 J. Clin. Endocrinol. Metab.Home page
B. Van Zaane, E. Nur, A. Squizzato, O. M. Dekkers, M. B. Twickler, E. Fliers, V. E. A. Gerdes, H. R. Buller, and D. P. M. Brandjes
Hypercoagulable State in Cushing's Syndrome: A Systematic Review
J. Clin. Endocrinol. Metab., August 1, 2009; 94(8): 2743 - 2750.
[Abstract] [Full Text] [PDF]

Home page
 J. Clin. Endocrinol. Metab.Home page
D. L. Batista, E. H. Oldfield, M. F. Keil, and C. A. Stratakis
Postoperative Testing to Predict Recurrent Cushing Disease in Children
J. Clin. Endocrinol. Metab., August 1, 2009; 94(8): 2757 - 2765.
[Abstract] [Full Text] [PDF]

Home page
 Eur J EndocrinolHome page
F Castinetti, M Fassnacht, S Johanssen, M Terzolo, P Bouchard, P Chanson, C Do Cao, I Morange, A Pico, S Ouzounian, et al.
Merits and pitfalls of mifepristone in Cushing's syndrome
Eur. J. Endocrinol., June 1, 2009; 160(6): 1003 - 1010.
[Abstract] [Full Text] [PDF]

Home page
 J. Clin. Endocrinol. Metab.Home page
M. Boscaro, W. H. Ludlam, B. Atkinson, J. E. Glusman, S. Petersenn, M. Reincke, P. Snyder, A. Tabarin, B. M. K. Biller, J. Findling, et al.
Treatment of Pituitary-Dependent Cushing's Disease with the Multireceptor Ligand Somatostatin Analog Pasireotide (SOM230): A Multicenter, Phase II Trial
J. Clin. Endocrinol. Metab., January 1, 2009; 94(1): 115 - 122.
[Abstract] [Full Text] [PDF]

Home page
 J. Clin. Endocrinol. Metab.Home page
R. Pivonello, M. C. De Martino, P. Cappabianca, M. De Leo, A. Faggiano, G. Lombardi, L. J. Hofland, S. W. J. Lamberts, and A. Colao
The Medical Treatment of Cushing's Disease: Effectiveness of Chronic Treatment with the Dopamine Agonist Cabergoline in Patients Unsuccessfully Treated by Surgery
J. Clin. Endocrinol. Metab., January 1, 2009; 94(1): 223 - 230.
[Abstract] [Full Text] [PDF]

Home page
 J. Clin. Endocrinol. Metab.Home page
C. A. Carrasco, J. Coste, L. Guignat, L. Groussin, M. A. Dugue, S. Gaillard, X. Bertagna, and J. Bertherat
Midnight Salivary Cortisol Determination for Assessing the Outcome of Transsphenoidal Surgery in Cushing's Disease
J. Clin. Endocrinol. Metab., December 1, 2008; 93(12): 4728 - 4734.
[Abstract] [Full Text] [PDF]


HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH
Endocrinology Endocrine Reviews J. Clin. End. & Metab.
Molecular Endocrinology Recent Prog. Horm. Res. All Endocrine Journals
Copyright © 2008 by The Endocrine Society