Treatment Options for Stage IV Adrenocortical Carcinoma (ACC)

Treatment options for stage IV ACC include:

Temporary palliation of disseminated ACC can sometimes be achieved with the chemotherapeutic agent mitotane. Although measurable partial remissions are unusual and are reported in only 20% to 30% of patients, palliation of hormone symptoms is commonly observed. Prolonged treatment with mitotane, however, is often limited by gastrointestinal and neurological toxicity. Local recurrences and selected sites of metastatic disease can sometimes be palliated surgically or with radiation therapy.[1,2]

Two other cytotoxic chemotherapy regimens may be effective and have been compared in a phase III clinical trial:[1]

Clinical trials of other chemotherapy regimens are ongoing.

Many patients with functioning tumors who receive treatment experience reduced hormone production. In patients with increased hormone production, antisteroidogenic drugs, such as ketoconazole and metyrapone, and steroid receptor antagonists, such as spironolactone and mifepristone, should be considered.

Clinical trials, including phase I and II trials of newer chemotherapeutic and biological agents, are appropriate and should be considered.