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Adrenocortical carcinoma: current treatment options.
Giuseppina De Filpo1, Massimo Mannelli, Letizia Canu
1Department of Experimental and Clinical Biomedical Sciences, University of Florence, Florence, Italy.
Adrenocortical carcinoma (ACC) treatment relies on surgery, with adjuvant mitotane improving survival for high-risk patients. Chemotherapy options exist for advanced ACC, while targeted therapies are under investigation.
Area of Science:
- Oncology
- Endocrinology
Background:
- Adrenocortical carcinoma (ACC) is a rare endocrine malignancy with a poor prognosis.
- Current treatment strategies are limited, necessitating research into novel therapeutic approaches.
Purpose of the Study:
- To review current and future treatment options for adrenocortical carcinoma (ACC).
- To highlight the role of surgery, adjuvant therapy, and chemotherapy in ACC management.
Main Methods:
- Review of current literature on adrenocortical carcinoma (ACC) treatment.
- Analysis of recent findings on adjuvant mitotane, chemotherapy regimens, and emerging targeted therapies.
Main Results:
- Radical surgery is the only curative option for ACC.
- Adjuvant mitotane improves overall survival in high-risk patients, with therapeutic drug monitoring crucial for efficacy.
- Etoposide, doxorubicin, cisplatin with mitotane (EDP-M) is a first-line option for inoperable ACC; gemcitabine, capecitabine, and streptozotocin are second-line choices.
- Temozolomide serves as a third-line option, but targeted therapies have shown unsatisfactory results to date.
Conclusions:
- Adjuvant mitotane is recommended for high-risk ACC patients post-surgery.
- The EDP-M regimen is standard for inoperable ACC, with limited data on subsequent lines of therapy.
- Ongoing clinical trials are evaluating tyrosine kinase and immune checkpoint inhibitors for ACC treatment.
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