期刊
NATURE CLINICAL PRACTICE ENDOCRINOLOGY & METABOLISM
卷 3, 期 11, 页码 748-757出版社
NATURE PUBLISHING GROUP
DOI: 10.1038/ncpendmet0648
关键词
adrenal tumors; Cushing's syndrome; phosphodiesterases; protein kinase A; tumor-suppressor genes
资金
- Intramural NIH HHS Funding Source: Medline
Adrenocortical causes of Cushing's syndrome include the following: common cortisol-producing adenomas, which are usually isolated (without associated tumors) and sporadic (without a family history); rare, but often clinically devastating, adrenocortical carcinomas; and a spectrum of adrenocorticotropin-independent, and almost always bilateral, hyperplasias, which are not rare, and are the most recently recognized cause. The majority of benign lesions of the adrenal cortex seem to be linked to abnormalities of the cyclic AMP signaling pathway, whereas cancer is linked to aberrant expression of insulin-like growth factor II, tumor protein p53 and related molecules. In this article, we propose a new clinical classification and nomenclature for the various forms of adrenocorticotropin-independent adrenocortical hyperplasias that is based on their histologic and genetic features. We also review the molecular genetics of adrenocortical tumors, including recent discoveries relating to the role of phosphodiesterase 11A. This is a timely Review because of recent advances in the clinical and molecular understanding of these diseases.
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