Genetics of adrenal tumors associated with Cushing's syndrome: a new classification for bilateral adrenocortical hyperplasias.
Nat Clin Pract Endocrinol Metab
; 3(11): 748-57, 2007 Nov.
Article
en En
| MEDLINE
| ID: mdl-17955016
ABSTRACT
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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Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Neoplasias de las Glándulas Suprarrenales
/
Síndrome de Cushing
Tipo de estudio:
Risk_factors_studies
Límite:
Animals
/
Humans
Idioma:
En
Revista:
Nat Clin Pract Endocrinol Metab
Asunto de la revista:
ENDOCRINOLOGIA
/
METABOLISMO
Año:
2007
Tipo del documento:
Article
País de afiliación:
Estados Unidos