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Treatment of Adrenocorticotropin-Dependent Cushing’s Syndrome: A Consensus Statement

Neuroendocrine Clinical Center, Massachusetts General Hospital (B.M.K.B., A.K., B.S.), Boston, Massachusetts 02114; Department of Endocrinology (A.B.G.), St. Bartholomew’s Hospital, London EC1A 7BE, United Kingdom; Division of Medical Sciences (P.M.S.), University of Birmingham, Queen Elizabeth Hospital, Birmingham B29 6JD, United Kingdom; Department of Medicine (S.M.), Cedars Sinai Medical Center, Los Angeles, California 90048; Endocrinology Department (X.B., J.B.), Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Faculté de Médecine Paris Descartes, 75014 Paris, France; Neurochirurgische Klinik (M.B.), Universität Erlangen, 91054 Nürnberg, Germany; Dipartimento di Endocrinologia ed Oncologia Molecolare e Clinica (A.C.), Università degli Studi di Napoli Federico II, 80131 Napoli, Italy; Department of Endocrinology (A.R.H.), University Medical Center Nijmegen, 6000500 HB Nijmegen, The Netherlands; Department of Internal Medicine (L.J.H.), Division of Endocrinology, Erasmus Medical Center, 3000 CA Rotterdam, The Netherlands; Division of Endocrinology (A.L.), Department of Medicine, Centre Hospitalier de l’Université de Montréal, Montréal, Quebec H2W 1T8, Canada; Altnagelvin Area Hospital (J.R.L), Londonderry BT47 6SB, United Kingdom; The Medical School (J.N.-P.), University of Sheffield, Sheffield S10 2TN, United Kingdom; Reproductive Biology and Medicine Branch (L.K.N.), National Institutes of Health, Bethesda, Maryland 20892; Division of Endocrinology (S.P.), University of Essen, 45122 Essen, Germany; Department of Statistical Sciences (N.S.), University of Padova, 35122 Padova, Italy; Department of Endocrinology (G.K.S.), Max Planck Institute of Psychiatry, 80804 Munich, Germany; University of Virginia Health System (M.L.V.), Charlottesville, Virginia 22903; Department of Endocrinology (J.A.H.W.), Oxford Centre for Diabetes, Endocrinology and Metabolism, Churchill Hospital, Oxford OX3 7LJ, United Kingdom; and Division of Endocrinology (M.B), Institute of Internal Medicine, Polytechnic University of Marche, 60126 Ancona, Italy

Address all correspondence and requests for reprints to: Marco Boscaro, M.D., Institute of Internal Medicine, Division of Endocrinology, School of Medicine, Polytechnic University of Marche, 60020 Torrette, Ancona, Italy. E-mail: m.boscaro{at}univpm.it.

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.

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 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.