title: [Recent progress in the treatment of Cushing's disease].
abstract: Transsphenoidal surgery, possibly through the endoscopic approach, remains the first line treatment. Opposing cortisol action with mifepristone proved efficacious in some individual cases but but with major monitoring difficulties. Combined treatment with three anticortisolic drugs (metyrapone, ketokonazole, O,p'DDD) is particularly attractive in severe cases. The Nelson's syndrome has been revisited, and the corticotroph tumor progression should rather be cautiously assessed after bilateral adrenalectomy. Two molecules potentially act directly to suppress the ACTH secretion by the corticotroph adenoma: agonists of the D2 Dopamine receptor and of the somatostatin receptor type 5. Their efficacy remains modest (20 to 30% of the patients actually normalize urinary cortisol). Pituitary radiotherapy can be efficiently performed by stereotaxic approach.
authors.affiliation: Service des maladies endocriniennes et métaboliques, centre de référence des maladies rares de la surrénale, faculté de médecine Paris-Descartes, université Paris, hôpital Cochin, France. xavier.bertagna@cch.aphp.fr
authors.forename: Xavier
authors.initials: X
authors.lastname: Bertagna
authors.forename: Laurence
authors.initials: L
authors.lastname: Guignat
mesh_terms: D049913:ACTH-Secreting Pituitary Adenoma / Q000150:complications / Q000601:surgery; D000236:Adenoma / Q000150:complications / Q000601:surgery; D004704:Endocrinology / Q000379:methods* / Q000639:trends*; D006727:Hormone Antagonists / Q000627:therapeutic use; D006801:Humans; D006854:Hydrocortisone / Q000037:antagonists & inhibitors; D008954:Models, Biological; D019635:Neurosurgical Procedures / Q000379:methods / Q000706:statistics & numerical data; D047748:Pituitary ACTH Hypersecretion / Q000150:complications / Q000628:therapy*; D011878:Radiotherapy / Q000706:statistics & numerical data
chemical_list: D006727:Hormone Antagonists; D006854:Hydrocortisone