WNT pathway deregulation in adrenal cortex tumorigenesis. (October 2019)

• Record Type: Journal Article
• Authors: Wilmouth, James; Olabe, Julie; Roucher-Boulez, Florence; Val, Pierre
• Is Part Of: Current opinion in endocrine and metabolic research. Volume 8(2019)
• Page Start: 174
• Page End: 182
• Publication Date: 2019-10
• Subjects: WNT signaling -- Beta-catenin -- Adrenal cortex -- Tumors -- Adenoma -- Carcinoma; Endocrine glands -- Diseases -- Periodicals; Metabolism -- Disorders -- Periodicals; Endocrine System Diseases; Metabolic Diseases; Endocrine glands -- Diseases; Metabolism -- Disorders; Periodical; Electronic journals; Periodicals; Electronic journals; 616.4
• DOI: 10.1016/j.coemr.2019.08.016
• Languages: English
• ISSNs: 2451-9650

Abstract

Abstract: Since 2005 and the discovery of canonical Wingless iNTegration 1 (WNT) pathway activation in adrenocortical tumors, a large body of work has identified the diverse roles of this signaling pathway in adrenal homeostasis and disease. This review focuses on WNT pathway deregulation in benign and malignant adrenal cortex tumors. It covers the role of CTNNB1 and APC alterations and provides data on the newly identified regulator ZNRF3. It raises questions on the mechanisms that may underlie the broad effects of WNT signaling pathway activation in adrenal tumorigenesis, which range from development of benign nonsecreting tumors to aggressive cortisol-producing carcinomas. Altogether it highlights the central role of this pathway in the adrenal, emphasizing the need for therapeutic targeting in adrenal diseases.