Abstract
Adrenocortical carcinomas (ACC) are rare tumors with unfavorable prognosis; they may be functional (hormone secreting) or nonfunctional. The estimated incidence is 1–2 per million per population and the overall 5-year survival is less than 40 %. Surgery is the mainstay of treatment in patients with localized disease, and metastasectomy can be performed in selected cases. Mitotane is a derivative of the insecticide dichlorodiphenyltrichloroethane and has been used in adjuvant, locally advanced, and metastatic setting in the treatment of ACC. Adjuvant therapy with mitotane after surgical excision is recommended in high-risk cases. In metastatic or unresectable disease, mitotane can be used alone or in combination with chemotherapy. Radiotherapy is usually utilized with palliative intent; recent retrospective studies also showed benefit of postoperative radiotherapy. Targeted therapies with tyrosine kinase inhibitors, vascular endothelial growth factor receptor inhibitors, or mTOR inhibitors alone or in combination are still under investigation.
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Orhan, B., Dizdar, O., Yalcin, S. (2015). Diagnosis and Management of Adrenocortical Carcinomas. In: Yalcin, S., Öberg, K. (eds) Neuroendocrine Tumours. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-45215-8_23
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