Contemporary preclinical human models of adrenocortical carcinoma

Adrenocortical carcinoma (ACC) is an uncommon and heterogeneous disease and may present differently in children and adults. Management of ACC is dependent on disease stage and complete surgical resection is the only potentially curative treatment. The first and most extensively used adrenocortical cancer cell line, as model system to examine mechanisms controlling normal and pathologic function of adrenal cortex, was initially isolated in 1980. Although NCI-H295 maintained steroid capabilities and adrenocortical characteristics, the lack of new cell lines and animal models of ACC has hampered the progress and development of new therapies. In this review we provide description of cellular and patient-derived tumor xenograft (PDTX) models of ACC generated for the elucidation of the underlying pathogenic mechanisms and preclinical functional studies for this aggressive disease.