Genetic, molecular and clinical features of familial isolated pituitary adenomas.
Horm Res
; 71 Suppl 2: 116-22, 2009 Apr.
Article
em En
| MEDLINE
| ID: mdl-19407507
ABSTRACT
Pituitary adenomas occur in a familial setting in about 5% of all cases, and over half of these are due to multiple endocrine neoplasia type 1 (MEN1) and Carney's complex (CNC). Non-MEN1/CNC familial pituitary tumours of all tumour phenotypes, known as familial isolated pituitary adenomas (FIPA), were first described in the late 1990s. Clinical features of FIPA differ from those of sporadic pituitary adenomas, as patients with FIPA have a younger age at diagnosis and larger tumours. About 15% of patients with FIPA have mutations in the aryl hydrocarbon receptor-interacting protein gene (AIP), which indicates that FIPA may have a diverse genetic pathophysiology. This article describes the clinical features of FIPA, the tumour pathologies found in this setting and the genetic/molecular data that have recently been reported in FIPA.
Texto completo:
1
Bases de dados:
MEDLINE
Assunto principal:
Neoplasias Hipofisárias
/
Doenças Genéticas Inatas
Limite:
Animals
/
Humans
Idioma:
En
Revista:
Horm Res
Ano de publicação:
2009
Tipo de documento:
Article
País de afiliação:
Bélgica