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