Reference : Update on the treatment of pituitary adenomas: familial and genetic considerations.
Scientific journals : Article
Human health sciences : Endocrinology, metabolism & nutrition
http://hdl.handle.net/2268/62547
Update on the treatment of pituitary adenomas: familial and genetic considerations.
English
Daly, Adrian [Université de Liège - ULiège > Département des sciences cliniques > Endocrinologie >]
Beckers, Albert mailto [Université de Liège - ULiège > Département des sciences cliniques > Endocrinologie >]
Nov-2008
Acta Clinica Belgica
Acta Clinica Belgica
63
6
418-424
Yes (verified by ORBi)
0001-5512
Bruxelles
Belgique
[en] Acromegaly ; Adenoma/therapy ; Animals ; Humans ; Intracellular Signaling Peptides and Proteins/genetics ; Loss of Heterozygosity ; Multiple Endocrine Neoplasia Type 1/complications/genetics ; Mutation ; Pituitary Neoplasms/drug therapy/etiology/genetics ; Prolactinoma/drug therapy/genetics
[en] Clinically-relevant pituitary adenomas occur with a prevalence of approximately 1 per 1000 population in Belgium. Pituitary adenomas that occur in families are likely to have an important genetic pathophysiological basis. Currently about 5% of all pituitary adenoma cases have a family history of pituitary adenomas, classically due to multiple endocrine neoplasia type 1 (MEN1) and Carney complex (CNC). Over the last decade we have described non-MEN1/CNC familial pituitary tumours that include all tumour phenotypes, a condition named 'familial isolated pituitary adenoma' (FIPA). Clinical features of FIPA differ from those of sporadic pituitary adenomas in that patients with FIPA are often younger and have larger tumours at diagnosis. Approximately 15% of FIPA patients have mutations in the aryl hydrocarbon receptor interacting protein gene (AIP), which indicates that FIPA may have a diverse genetic pathophysiology. In this review we examine new findings on the epidemiology of pituitary adenomas and we review familial causes of pituitary adenomas with a particular emphasis on modern clinical testing. In addition, the clinical and genetic features of FIPA are described as FIPA represents a useful framework to study the features of pituitary adenomas that occur in a familial setting.
Researchers ; Professionals
http://hdl.handle.net/2268/62547

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