Mouse models of adrenocortical tumors. (5th February 2016)

• Record Type: Journal Article
• Authors: Basham, Kaitlin J.; Hung, Holly A.; Lerario, Antonio M.; Hammer, Gary D.
• Is Part Of: Molecular and cellular endocrinology. Volume 421(2016)
• Page Start: 82
• Page End: 97
• Publication Date: 2016-02-05
• Subjects: Adrenal cortex -- Adrenal tumor model -- Adrenocortical carcinoma -- Adrenal hyperplasia -- Gonadectomy-induced tumorigenesis -- Spontaneous adrenal tumor; Endocrinology -- Periodicals; Molecular biology -- Periodicals; Cytology -- Periodicals; Endocrinology -- Periodicals; Hormones -- Periodicals; Endocrinologie -- Périodiques; Cytology; Endocrinology; Molecular biology; Periodicals; 573.4
• DOI: 10.1016/j.mce.2015.11.031
• Languages: English
• ISSNs: 0303-7207

Abstract

Abstract: The molecular basis of the organogenesis, homeostasis, and tumorigenesis of the adrenal cortex has been the subject of intense study for many decades. Specifically, characterization of tumor predisposition syndromes with adrenocortical manifestations and molecular profiling of sporadic adrenocortical tumors have led to the discovery of key molecular pathways that promote pathological adrenal growth. However, given the observational nature of such studies, several important questions regarding the molecular pathogenesis of adrenocortical tumors have remained. This review will summarize naturally occurring and genetically engineered mouse models that have provided novel tools to explore the molecular and cellular underpinnings of adrenocortical tumors. New paradigms of cancer initiation, maintenance, and progression that have emerged from this work will be discussed. Highlights: Summary of spontaneous/genetic models of adrenal tumors and related human pathology. Models associated with rare genetic syndromes and adrenal hyperplasia are discussed. Gonadectomy promotes adrenal neoplasia and aberrant progenitor cell specification. Mouse models have studied Wnt, IGF2 and p53 dysregulation in adrenal tumorigenesis. Novel models are needed to recapitulate the high complexity of human adrenal tumors.