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Review
. 2009 Sep 20;27(27):4619-29.
doi: 10.1200/JCO.2008.17.2775. Epub 2009 Aug 10.

Management of Endocrine Manifestations and the Use of Mitotane as a Chemotherapeutic Agent for Adrenocortical Carcinoma

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Free PMC article
Review

Management of Endocrine Manifestations and the Use of Mitotane as a Chemotherapeutic Agent for Adrenocortical Carcinoma

Irina Veytsman et al. J Clin Oncol. .
Free PMC article

Abstract

Adrenal cortical carcinoma (ACC) is a rare malignancy in which patients have poor overall 5-year survival. Patients with ACC can present with symptoms of hormone excess, including Cushing's syndrome, virilization, feminization, or--less frequently--hypertension with hypokalemia. In many patients with ACC, advanced disease at presentation precludes surgery or is followed by local relapse or distant metastatic disease that cannot be managed surgically. In these instances, chemotherapy is often tried, but its limited efficacy all too often leaves the problem of persistent hormonal excess. Physicians who treat patients with ACC and severe hypercortisolism should recognize that uncontrolled hormone production is a malignant disease, which has severe consequences that require aggressive management. Because chemotherapy benefits only a small percentage of patients, steroidogenesis inhibitors, including mitotane, ketoconazole, metyrapone, and etomidate, should be used singly or in combination even as chemotherapy is administered. Diligent management with frequent adjustments is required, especially in patients with chemotherapy-refractory tumors that continue to grow. In the absence of randomized, controlled trials, adjuvant use of mitotane remains controversial, although the authors of a recent case-control study argue for its use. Despite difficulty administering effective doses, most clinicians agree that mitotane should be used if the tumor cannot be removed surgically or should be used as adjuvant therapy if there is a high likelihood of recurrence. The option of long-term monotherapy is restricted to patients who tolerate mitotane and either experience a clinical response or are at high risk for recurrence. Recommendations are provided to help manage patients with this difficult disease and to improve the quality of their lives.

Conflict of interest statement

Authors' disclosures of potential conflicts of interest and author contributions are found at the end of this article.

Figures

Fig 1.
Fig 1.
Steroid biosynthetic pathway in the normal human adrenal gland. Enzymes required for steroidogenesis are drug treatment targets.
Fig A1.
Fig A1.
Radiologic imaging of adrenocortical cancer. CT, computed tomography; MRI, magnetic resonance imaging.

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