title: [Adrenal cortical carcinoma: advances in the pathophysiology and management of this malignancy].
abstract: Adrenal cortical carcinoma is a rare malignancy, with only one or two new cases being diagnosed per million subjects per year. The prognosis is dismal, with less than 30% survival at five years. The best chances of cure are obtained when a "localized" tumor can undergo "complete" surgical removal. Most often, however, the diagnosis is made when the tumor is already invasive and non secretory Clinical, hormonal and imaging features, including 18-fluorodeoxyglucose PET scan, can provide strong evidence of malignancy and indicate open surgical excision in expert hands. Recent advances in the genetics of adrenal cortical carcinomas have identified molecular factors that can be used as diagnostic and prognostic markers. These markers are more valuable than classical staging and histology (Weiss score). A better understanding of the pathophysiology of these tumors is required in order to develop targeted therapies.
authors.affiliation: Service des Maladies Endocriniennes et Métaboliques, Hôpital Cochin, Paris, Faculté de médecine Paris-Descartes, Université Paris 5.
authors.forename: Xavier
authors.initials: X
authors.lastname: Bertagna
authors.forename: Lionel
authors.initials: L
authors.lastname: Groussin
authors.forename: Rossella
authors.initials: R
authors.lastname: Libe
authors.forename: Jérôme
authors.initials: J
authors.lastname: Bertherat
mesh_terms: D000306:Adrenal Cortex Neoplasms / Q000175:diagnosis / Q000453:epidemiology / Q000235:genetics / Q000628:therapy*; D002277:Carcinoma / Q000175:diagnosis / Q000453:epidemiology / Q000235:genetics / Q000628:therapy*; D006801:Humans; D009367:Neoplasm Staging; D011379:Prognosis