Context: Adrenocortical carcinoma (ACC) is a rare cancer but one that has devastating consequences for affected patients. Surgery is the mainstay of therapy, although the high frequency of metastatic disease implies that it is frequently noncurative. Traditional cytotoxic chemotherapy for ACC has generally produced disappointing responses, implying the need for the new therapies for this disease. Evidence Acquisition: Review articles and primary literature were identified by extensive PubMed

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... rching to obtain papers evaluating the current state of knowledge regarding ACC, as well as assessing the development of new therapeutic modalities for the treatment of cancer. When needed, additional articles were identified from the reference lists of the papers obtained from the primary screen. Evidence Synthesis: Multiple new modalities that may enhance the future treatment of ACC were identified. They include the following: combating drug resistance, targeting tumor vasculature, inhibiting signaling pathways with small molecules, and using gene and/or immunotherapy. This review provides a brief summary of the progress and prospects of each of these modalities and focuses on emerging data and treatments that may alter the course of this disease within the next few years. Conclusions: Despite the current grim outlook, the recent applications of emerging technology to the study of ACC and the development of newer, "targeted" therapies for cancer suggest the possibility of a new hope for patients with this disease, although these therapies will need to be evaluated by rigorous clinical trials to verify their effectiveness. (J Clin Endocrinol Metab 91: 14 -21, 2006)