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[Recent data in adrenocortical tumorigenesis]

Ann Endocrinol (Paris). 2009 Sep ;70 Suppl 1:S20-5
[Recent data in adrenocortical tumorigenesis]
Crand A, Borson-Chazot F, Brue T.

Adrenocortical carcinomas are rare tumors characterized by an aggressive behaviour with a 5-year survival rate below 30%. Until now, surgery is the only curative treatment for tumors confined to the adrenal gland and there is a lack of an effective medical treatment for invasive or metastatic tumors due to the poor knowledge of the mechanisms underlying adrenocortical malignancy. Moreover, histopathology is sometimes insufficient to establish an accurate diagnosis between a benign and a malignant adrenal tumor and a poor indicator of prognosis. In the last decade, the study of rare genetic syndromes associated with adrenocortical carcinomas and the identification of genetic alterations in adrenal tumors has improved our understanding of the pathogenesis of adrenal tumors. The development of molecular predictors of malignancy and of survival could help for histological diagnosis and determination of prognosis. These significant advances are essential to improve adrenocortical carcinoma management. This review summarizes recent advances in the understanding and management of adrenocortical tumors.


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