Purpose of Review
Adrenocortical carcinoma (ACC) is a rare endocrine malignancy typically with poor prognosis. This review aims to summarize the current knowledge regarding the clinical management of ACC.
Surgery remains the cornerstone for localized ACC management. In more advanced cases, debulking surgery when feasible can help with hormonal control and may allow the initiation of systemic therapy. Over the last few years, our understanding of ACC molecular pathogenesis has expanded with no significant change in treatment options. Platinum-based chemotherapy is the gold standard in metastatic ACC despite suboptimal efficacy. Tyrosine kinase inhibitor use did not result in meaningful benefit in ACC patients. Multiple clinical trials are currently exploring the role of immunotherapy in ACC.
Despite the remarkable improvement in our understanding of the molecular signature and pathways in ACC, this knowledge did not yield a major breakthrough in management of advanced ACC. Multi-institutional and international collaborations are needed to identify promising treatments and new therapeutic targets to improve the care of ACC patients.
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Conflict of Interest
Sina Jasim declares that she has no conflict of interest. Mouhammed Amir Habra has received research funding from Exelixis and has received compensation from HRA Pharma and Eisai for service as a consultant.
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Jasim, S., Habra, M.A. Management of Adrenocortical Carcinoma. Curr Oncol Rep 21, 20 (2019). https://doi.org/10.1007/s11912-019-0773-7
- Adrenocortical carcinoma
- Targeted therapy
- Genomic profiling