Abstract
Adrenocortical carcinoma (ACC) is a rare disease with poor prognosis.Patients usually present with signs of steroid hormone excess (e.g.Cushing's syndrome, Conn's syndrome, virilization) or an abdominal mass.In functional cases, Cushing's syndrome is the most frequent presentation.Mitotane is still the most important single drug for ACC treatment, and it plays a role in adjuvant therapy postoperatively as well as in advanced disease.In advanced ACC, a combination of mitotane with etoposide, doxorubicin, cisplatin or streptozotocin with mitotane may be used.Targeted therapies, such as insulin-like growth factor Ⅰ (IGF- Ⅰ)receptor inhibitor and multiple kinase inhibitors like sunitinib and sorafenib hold great promises. Key words: Adrenocortical carcinoma; Mitotane; Cytotoxic chemotherapy; Targeted therapy
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