Adrenal cortical tumors and multiple endocrine neoplasia-related syndromes.
Minerva Endocrinol
; 34(2): 123-35, 2009 Jun.
Article
em En
| MEDLINE
| ID: mdl-19471237
ABSTRACT
Relatively frequent, adrenal masses include a multitude of different tumor types uni- or bilateral hyperplasias, adenomas, and the rare entity of adrenocortical carcinomas. With significant progress in our appreciation of their underlying molecular pathomechanisms and from analysis of affected individuals and their families, a number of inherited diseases and tumor syndromes have been linked to adrenocortical tumorigenesis. These syndromes and diseases include the Carney complex, the McCune-Albright syndrome, multiple endocrine neoplasia type 1, familial adenomatosis coli, congenital adrenal hyperplasia, familial forms of primary aldosteronism, the Beckwith-Wiedemann syndrome, and the Li-Fraumeni syndrome. The key to successful management of these syndromes is identification of patients harboring adrenal tumors within the context of hereditary diseases, since diagnostic procedures, therapy and follow-up may significantly differ from the management of sporadic, isolated adrenal tumors. This review explores the underlying genetic defects, diagnosis and therapy of the major heritable tumor syndromes associated with adrenocortical tumorigenesis.
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Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Neoplasia Endócrina Múltipla
/
Neoplasias do Córtex Suprarrenal
/
Mutação
Tipo de estudo:
Diagnostic_studies
/
Prognostic_studies
Limite:
Humans
Idioma:
En
Revista:
Minerva Endocrinol
Ano de publicação:
2009
Tipo de documento:
Article
País de afiliação:
Alemanha