Adrenocortical cancer is a rare condition and chemotherapy is relatively unexplored. Studies involve small case series, single-patient trials and anecdotal unpublished communications. Compounds that have been used include established chemotherapeutic agents for treatment of other solid tumors, as well as more recent compounds, used in single cases. These agents include streptozotocin, gemcitabine, navelbine, docetaxel and taxol. The best evidence is for streptozotocin. Mitotane is the only FDA approved drug but it has a narrow therapeutic window. Based on the results of a case control study, mitotane is being explored as adjuvant therapy. An ongoing randomized international trial aims to define the best combination chemotherapy plus mitotane regimen. Genetic and biological studies have identified molecular targets for specific targeted drugs such as IGF receptor inhibitors and antiangiogenetic drugs. Phase II trials are exploring the activity of these drugs either alone or in combination with chemotherapy.However, available evidence does not allow for any conclusions on the efficacy of these agents in ACC.
Of the newer drugs, there is a case report, one for Nexavar and one for Sutent. This clearly is insufficient to consider these agents to be adequately supported.
September 2008, Vol. 13, No. 3, Pages 497-509 , DOI 10.1517/14728126.96.36.1997