In recent years, several mouse models have been established for
characterization of the molecular pathways involved in adrenocortical
tumorigenesis. Adrenal tumors develop in genetically susceptible mouse
strains after prepubertal gonadectomy, in mice transgenic with oncogenes
(simian virus 40 T antigen), several gene knockouts (such as inhibin or
conditional Gata6^F/F), and in mice overexpressing
transcription factor GATA binding protein 4. The gonadal rest-type
adrenal tumor phenotype is regulated by gonadotropins, mainly
luteinizing hormone. Luteinizing hormone/chorionic hormone receptor and
gonadotropin-releasing hormone receptor expression has been found in
human adrenocortical carcinoma, as well as in several mouse adrenal
tumor/adrenocarcinoma models. This mini-review will address recent
advancements in this research topic with respect to the molecular basis
of adrenocortical tumorigenesis, the clinical relevance of these tumor
models, and the potential for future targeted treatment strategies.
Furthermore, the ectopic expression of the luteinizing hormone/chorionic
hormone receptor or gonadotropin-releasing hormone receptor may open up
options for targeted therapy approaches.