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Adrenocortical Carcinoma Treatment (PDQ®)

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Stage III Adrenocortical Carcinoma

Standard treatment options:

  • Complete surgical removal of the tumor, with or without regional lymph node dissection, is the treatment of choice for patients with stage III adrenocortical carcinomas. The treatment of patients who have tumors with local invasion, but without clinically enlarged regional lymph nodes, is complete surgical removal as for stage I and stage II tumors. For those with enlarged regional lymph nodes, a lymph node dissection should be included in the procedure. These patients are at a high risk of disease recurrence and should be considered for enrollment in a clinical trial.

Treatment options under clinical evaluation:

  1. Clinical trials are appropriate for newly diagnosed patients when possible.
  2. Radiation therapy (approximately 50 to 70 Gy given over a period of 4 weeks) may be given to patients with localized but unresectable tumors.[1]
  3. For patients unable to undergo complete resection, mitotane in doses as high as 10 to 12 g per day to achieve a blood level of 14 to 20 mg/L should be considered. This adrenolytic drug produces useful clinical responses in about 20% to 30% of patients with measurable tumor burden.[2,3]

    Two other cytotoxic chemotherapy regimens are suggested to be effective and have been compared in a phase III trial:[2]

    • Streptozotocin plus mitotane.
    • 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.

    The role of mitotane as adjuvant therapy after complete tumor resection is still a matter of debate but should be discussed with the patient. In the case of complete resection, the role for adjuvant mitotane and radiation therapy is the same as for stage I and stage II adrenocortical carcinoma.

Current Clinical Trials

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with stage III 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.


  1. Percarpio B, Knowlton AH: Radiation therapy of adrenal cortical carcinoma. Acta Radiol Ther Phys Biol 15 (4): 288-92, 1976. [PUBMED Abstract]
  2. Allolio B, Fassnacht M: Clinical review: Adrenocortical carcinoma: clinical update. J Clin Endocrinol Metab 91 (6): 2027-37, 2006. [PUBMED Abstract]
  3. 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.
  • Updated: November 15, 2012