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Adrenocortical Carcinomas: Molecular Pathogenesis, Treatment Options, and Emerging Immunotherapy and Targeted Therapy Approaches
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Adrenocortical Carcinomas: Molecular Pathogenesis, Treatment Options, and Emerging Immunotherapy and Targeted Therapy Approaches
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Journal Article
Adrenocortical Carcinomas: Molecular Pathogenesis, Treatment Options, and Emerging Immunotherapy and Targeted Therapy Approaches
2024
Overview
Abstract
Adrenocortical carcinoma (ACC) is a rare and aggressive malignancy in the advanced setting with poor prognosis. This narrative review provides an overview of the epidemiology of ACC and its molecular pathogenesis with a summary of the main involved signaling pathways. We then provide an update on the clinical presentation, diagnosis, and current management strategies of both localized and metastatic disease from a multidisciplinary perspective. We highlight the debate around the use of mitotane in the adjuvant setting and review the use of combination chemotherapy with etoposide, doxorubicin, and cisplatin. The review also focuses on emerging data providing hope for the use of immune checkpoint inhibitors and targeted therapies in ACC with a summary of ongoing trials.
This review reports on the epidemiology of adrenocortical carcinoma and its molecular pathogenesis. An update on clinical presentation, diagnosis, and current management strategies is provided, as well as emerging data on the use of immune checkpoint inhibitors and targeted therapies.
Publisher
Oxford University Press
Subject
Adrenal Cortex Neoplasms - drug therapy
/ Adrenal Cortex Neoplasms - genetics
/ Adrenal Cortex Neoplasms - pathology
/ Adrenal Cortex Neoplasms - therapy
/ Adrenocortical Carcinoma - drug therapy
/ Adrenocortical Carcinoma - pathology
/ Adrenocortical Carcinoma - therapy
/ Antineoplastic Combined Chemotherapy Protocols - pharmacology
/ Antineoplastic Combined Chemotherapy Protocols - therapeutic use
/ Humans
/ Immune Checkpoint Inhibitors - pharmacology
/ Immune Checkpoint Inhibitors - therapeutic use
/ Molecular Targeted Therapy - methods
/ Mutation
/ Yeast
