Adrenocortical Carcinoma: Review of the Pathologic Features, Production of Adrenal Steroids, and Molecular Pathogenesis

Adrenocortical carcinoma (ACC) is a malignant neoplasm often associated with an aggressive biological behavior. The histologic differentiation between ACC and adrenocortical adenoma (ACA) is largely determined by employing the Weiss criteria, although this classification may not apply to all the cases. Additionally, various genomic features of ACC could be an auxiliary mode to establish the diagnosis of ACC. Most ACC cases are hormonally functional, and immunohistochemical analysis of steroidogenic enzymes has provided pivotal information as to the analysis of intratumoral production of corticosteroids. This article summarizes the current status of the histopathological diagnosis, molecular pathogenesis, and hormonal features of ACC.