Genetics of adrenal tumors associated with Cushing's syndrome: a new classification for bilateral adrenocortical hyperplasias

摘要: Bilateral adrenocortical hyperplasias (BAHs) are one cause of adrenocorticotropin-independent Cushing's syndrome. Until recently BAHs were relatively unknown and thought to be infrequent. This article reviews the molecular genetics tumor development with an emphasis on BAHs. It also suggests a classification for that is based their histology. Adrenocortical causes syndrome include following: common cortisol-producing adenomas, which usually isolated (without associated tumors) sporadic family history); rare, but often clinically devastating, carcinomas; spectrum adrenocorticotropin-independent, almost always bilateral, hyperplasias, not most recognized cause. The majority benign lesions adrenal cortex seem linked abnormalities cyclic AMP signaling pathway, whereas cancer aberrant expression insulin-like growth factor II, protein p53 related molecules. In this article, we propose new clinical nomenclature various forms histologic genetic features. We review tumors, including recent discoveries relating role phosphodiesterase 11A. timely Review because advances in understanding these diseases.