Preclinical Models of Adrenocortical Cancer

SIMPLE SUMMARY: Adrenocortical cancer is a very rare form of endocrine cancer with dismal prognosis. Preclinical models such as cell lines, organoids, and mouse models are essential for both improving basic understanding of this disease and developing treatments. Herein, we review a currently available model for adrenocortical cancers, with a special focus on adrenocortical carcinoma. Recent developments in in vitro models have included cell and 3D culture models with improved recapitulation of the tumor microenvironment and genetics. We hope to improve visibility and access to these models through this review. ABSTRACT: Adrenocortical cancer is an aggressive endocrine malignancy with an incidence of 0.72 to 1.02 per million people/year, and a very poor prognosis with a five-year survival rate of 22%. As an orphan disease, clinical data are scarce, meaning that drug development and mechanistic research depend especially on preclinical models. While a single human ACC cell line was available for the last three decades, over the last five years, many new in vitro and in vivo preclinical models have been generated. Herein, we review both in vitro (cell lines, spheroids, and organoids) and in vivo (xenograft and genetically engineered mouse) models. Striking leaps have been made in terms of the preclinical models of ACC, and there are now several modern models available publicly and in repositories for research in this area.