Summary and Introduction
Adrenocortical carcinoma (ACC) is a rare neoplasm with poor prognosis. Patients present with signs of steroid hormone excess (e.g. Cushing's syndrome, virilization) or an abdominal mass. Tumour size at presentation (mean diameter at diagnosis > 10 cm) is the most important indicator of malignancy. In addition, computed tomography (CT) typically demonstrates an inhomogeneous adrenal lesion with irregular margins and variable enhancement of solid components after intravenous contrast media. Magnetic resonance imaging (MRI) is equally effective as CT and is particularly helpful to visualize invasion into large vessels. Complete tumour removal (R0 resection) offers by far the best chance for long-term survival and therefore surgery is the treatment of choice in stage I-III ACC. Despite tumour resection for cure most patients will eventually develop local recurrence or distant metastases. Thus adjuvant treatment options need to be evaluated in high-risk patients (e.g. radiation therapy of the tumour bed and/or chemotherapy). In tumour recurrence re-operation should always be considered. In metastatic disease (stage IV ACC) not amenable to surgery mitotane (o,p'DDD) remains the first-line therapy. Drug monitoring is needed for effective treatment aiming at concentrations between 14 and 20 mg/l. Patients not responding to mitotane may benefit from cytotoxic chemotherapy (23% partial remissions, 4% complete remissions). Only large prospective multicentre trials comparing different treatment options will allow to make systematic progress in the management of ACC.
Adrenocortical carcinoma (ACC) is a rare and highly malignant neoplasm with poor prognosis. The incidence is approximately 1-2 per million population per year (National Cancer Institute, 1975; Dackiw et al., 2001) leading to 0·2% of cancer deaths according to data from the USA (Wajchenberg et al., 2000). The age distribution is bimodal with a first peak in childhood and a higher second peak in the 4th to 5th decade (Luton et al., 1990; Wooten & King, 1993; Wajchenberg et al., 2000). In a recent review of 920 adult patients with ACC, the mean age at diagnosis was around 45 years (Wajchenberg et al., 2000). In a meta-analysis by Wooten & King (1993), including more than 1800 cases, ACC was somewhat more frequent in women (59%) than in men. Epidemiologic studies have suggested an increased risk of ACC with the use of oral contraceptives and smoking (Hsing et al., 1996). In addition, adrenal tumours including ACC have been associated with poorly treated congenital adrenal hyperplasia (Allolio, 2001).
Clin Endocrinol. 2004;60(3) © 2004 Blackwell Publishing
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