title: The Nelson's syndrome... revisited.
abstract: Adrenalectomy is a radical therapeutic approach to control hypercortisolism in some patients with Cushing's disease. However it may be complicated by the Nelson's syndrome, defined by the association of a pituitary macroadenoma and high ACTH secretion after adrenalectomy. This definition has not changed since the end of the fifties. Today the Nelson's syndrome must be revisited with new to criteria using more sensitive diagnostic tools, especially the pituitary magnetic resonance imaging. In this paper we will review the pathophysiological aspects of corticotroph tumor growth, with reference to the impact of adrenalectomy. The main epidemiological data on the Nelson's syndrome will be presented. More importantly, we will propose a new pathophysiological and practical approach to this question which attempts to evaluate the Corticotroph Tumor Progression after adrenalectomy, rather than to diagnose the Nelson's syndrome. We will discuss the consequences for the management of Cushing's disease patients after adrenalectomy, and will also draw some perspectives.
authors.affiliation: Université René Descartes, Endocrinology, Cochin Hospital, Paris 5, France.
authors.forename: Guillaume
authors.initials: G
authors.lastname: Assié
authors.forename: Hélène
authors.initials: H
authors.lastname: Bahurel
authors.forename: Jérôme
authors.initials: J
authors.lastname: Bertherat
authors.forename: Michèle
authors.initials: M
authors.lastname: Kujas
authors.forename: Paul
authors.initials: P
authors.lastname: Legmann
authors.forename: Xavier
authors.initials: X
authors.lastname: Bertagna
mesh_terms: D049913:ACTH-Secreting Pituitary Adenoma / Q000209:etiology / Q000503:physiopathology* / Q000628:therapy; D000315:Adrenalectomy / Q000009:adverse effects; D000324:Adrenocorticotropic Hormone / Q000378:metabolism; D000328:Adult; D003480:Cushing Syndrome / Q000503:physiopathology / Q000601:surgery; D018450:Disease Progression; D005260:Female; D006801:Humans; D009347:Nelson Syndrome / Q000453:epidemiology / Q000503:physiopathology / Q000628:therapy; D047748:Pituitary ACTH Hypersecretion / Q000503:physiopathology / Q000601:surgery; D010911:Pituitary Neoplasms / Q000209:etiology / Q000503:physiopathology* / Q000628:therapy; D015995:Prevalence
chemical_list: D000324:Adrenocorticotropic Hormone