Temporary palliation of disseminated adrenocortical carcinomas can sometimes be achieved with the chemotherapeutic agent mitotane. Although measurable partial remissions are unusual and are reported in only 20% to 30% of cases, palliation of hormone symptoms is commonly observed. Prolonged treatment with mitotane, however, is often limited by gastrointestinal and neurologic 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 are suggested to be effective and have been compared in a phase III trial:
Incidence and Mortality
The majority of tumors of the paranasal sinuses present with advanced disease, and cure rates are generally poor (≤50%). Squamous cell carcinoma (SCC) is the most frequent type of malignant tumor in the nose and paranasal sinuses (70%–80%). Papillomas are distinct entities that may undergo malignant degeneration. The cancers grow within the bony confines of the sinuses and are often asymptomatic until they erode and invade adjacent structures.[1,2,3]
Etoposide, doxorubicin, and cisplatin plus mitotane.
Furthermore, a substantial number of treated patients with functioning tumors will show diminution in hormone production. In cases of increased hormone production, antisteroidogenic drugs, such as ketoconazole and metyrapone, and steroid receptor antagonists, such as spironolactone and mifepristone, should be considered.
Clinical trials are appropriate and should be considered whenever possible because phase I and II trials evaluate newer chemotherapeutic and biologic agents.
Standard treatment options:
Chemotherapy with mitotane.
Chemotherapy with mitotane plus streptozotocin or mitotane plus etoposide, doxorubicin, and cisplatin is used as part of the NCT00924144 phase III trial.
Radiation therapy to bone metastases.
Surgical removal of localized metastases, particularly those that are functioning.
Treatment options under clinical evaluation:
Several new agents are currently under investigation for unresectable adrenocortical cancer, including the following:
Insulin-like growth factor 1 receptor–inhibitors.
Trials of other chemotherapy regimens are also ongoing.
Current Clinical Trials
Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with stage IV adrenocortical carcinoma. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
General information about clinical trials is also available from the NCI Web site.
Terzolo M, Ardito A, Zaggia B, et al.: Mitotane. In: Hammer GD, Else T, eds.: Adrenocortical Carcinoma: Basic Science and Clinical Concepts. New York, Springer, 2010, pp 369-82.
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May 28, 2015
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