Mouse models of adrenal tumorigenesis

Adrenocortical carcinomas (ACCs) are heterogeneous tumors with a poor prognosis. The rarity of this disorder causes a lack of treatment experience and material availability which is necessary to optimize existing treatments and to develop novel therapeutic strategies. Although surgery is still the treatment of choice, adjuvant therapies are urgently needed as the rate of recurrence for these tumors is high. In recent years molecular characterization of surgical tumor specimen has aided in the understanding of disease mechanisms and definition of therapeutic targets also in adrenocortical carcinoma. However, most of the functional properties of potential target molecules are still unpredictable from pure expression and sequence analysis. For functional studies of gene products, mouse models remain to be intensively utilized as an experimental system due to the similarity to humans with respect to genome organization, development and physiology. Here we give an overview on rodent models that have been described to either have adrenocortical tumors as part of their phenotype or have been utilized for therapeutic screens as adrenocortical tumor models.