Background: With a reported incidence of 1 to 2 cases per million, adrenocortical cancer (ACC) is a rare disease with poor prognosis. Age distribution shows two peaks: early childhood and between age 40 and 50 years, with females more frequently affected. Sequelae can include Cushing syndrome, virilization and hypertension or local symptoms consistent with abdominal obstruction. Although most cases of ACC are of sporadic origin, they may also occur as part of a congenital or familial disease in which the genetic abnormalities are well established. ACC can also be discovered incidentally in asymptomatic individuals. In sporadic ACC, some molecular modifications are commonly observed (i.e., overexpression of insulin-like growth factor II or vascular endothelial growth factor and somatic mutations of tumor protein 53). When surgical resection of the tumor is impossible or ineffective, chemotherapy with etoposide, doxorubicin and cisplatin plus mitotane or with streptozotocin plus mitotane is frequently used; however, the overall survival rates are disappointing.
Conclusions: Hormonal evaluation is essential to diagnose ACC and the prognosis depends on many factors. New treatments, such as insulin-like growth factor I receptor antibodies, tyrosine kinase inhibitors and other antiangiogenic compounds, are now being intensively investigated to identify better therapies for this extremely severe malignant neoplasia.
Copyright 2009 S. Karger AG, Basel.