Adrenocortical carcinoma: Diagnosis, prognostic classification and treatment of localized and advanced disease

Rossella Libé; Olivier Huillard

doi:10.1016/j.ctarc.2023.100759

Adrenocortical carcinoma: Diagnosis, prognostic classification and treatment of localized and advanced disease

Cancer Treat Res Commun. 2023:37:100759. doi: 10.1016/j.ctarc.2023.100759. Epub 2023 Sep 8.

Authors

Rossella Libé¹, Olivier Huillard²

Affiliations

¹ Service Endocrinologie, AP-HP, Hôpital Cochin, French National Network, ENDOCAN-COMETE, F-75014, Paris, France.
² Institut du Cancer Paris CARPEM, AP-HP, Department of medical oncology, Hôpital Cochin, F-75014, Paris, France. Electronic address: olivier.huillard@aphp.fr.

PMID: 37690343
DOI: 10.1016/j.ctarc.2023.100759

Abstract

Adrenocortical carcinoma (ACC) is a rare cancer with an estimated incidence of 0.7 to 2.0 cases per 1 million population per year in the United States. It is an aggressive cancer originating in the cortex of the adrenal gland with a poor prognosis. The 5-year survival rate is less than 15% among patients with metastatic disease. In this article, we review the epidemiology and pathogenesis of ACC, the diagnostic procedures, the prognostic classification of ACC, and the treatment options from localized and resectable forms to advanced disease detailing recent therapeutic developments such as immunotherapy and molecularly targeted therapy.

Keywords: Adrenocortical carcinoma; Diagnostic; Mitotane.

Publication types

Review

MeSH terms

Adrenal Cortex Neoplasms* / drug therapy
Adrenal Cortex Neoplasms* / therapy
Adrenocortical Carcinoma* / diagnosis
Adrenocortical Carcinoma* / therapy
Antineoplastic Combined Chemotherapy Protocols / therapeutic use
Humans
Immunotherapy
Prognosis