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The adrenocortical carcinoma (ACC) is a primary malignant tumor developed from the adrenal cortex, defined by a Weiss score≥3. Its prognosis is poor and depends mainly on the stage of the disease at diagnosis. Care is organized in France by the multidisciplinary expert centers of the national ENDOCA...

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Bibliographic Details
Published in:Bulletin du cancer Vol. 110; no. 10; pp. 707 - 730
Main Authors: Libé, Rossella, Haissaguerre, Magalie, Renaudin, Karine, Faron, Matthieu, Decaussin-Petrucci, Myriam, Deschamps, Fréderic, Gimenez-Roqueplo, Anne-Paule, Mirallié, Eric, Murez, Thibaut, Pattou, François, Rocher, Laurence, Taïeb, David, Savoie, Pierre Henri, Tabarin, Antoine, Bertherat, Jérôme, Baudin, Eric, De La Fouchardière, Christelle
Format: Journal Article
Language:English
Published: Elsevier 01-06-2023
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Summary:The adrenocortical carcinoma (ACC) is a primary malignant tumor developed from the adrenal cortex, defined by a Weiss score≥3. Its prognosis is poor and depends mainly on the stage of the disease at diagnosis. Care is organized in France by the multidisciplinary expert centers of the national ENDOCAN-COMETE "Adrenal Cancers" network, certified by the National Cancer Institute. This document updates the guidelines for the management of ACC in adults based on the most robust data in the literature. It's divided into 11 chapters: (1) circumstances of discovery; (2) pre-therapeutic assessment; (3) diagnosis of ACC; (4) oncogenetics; (5) prognostic classifications; (6) treatment of hormonal hypersecretion; (7) treatment of localized forms; (8) treatment of relapses; (9) treatment of advanced forms; (10) follow-up; (11) the particular case of ACC and pregnancy. R0 resection of all localized ACC remains an unmet need and it must be performed in expert centers. Flow-charts for the therapeutic management of localized ACC, relapse or advanced ACC are provided. It was written by the experts from the national ENDOCAN-COMETE network and validated by all French Societies involved in the management of these patients (endocrinology, medical oncology, endocrine surgery, urology, pathology, genetics, nuclear medicine, radiology, interventional radiology).
ISSN:0007-4551
1769-6917
DOI:10.1016/j.bulcan.2023.03.015