[Genetics of familial pituitary adenomas].
Pituitary adenomas were previously thought to be rare. However, a recent cross-sectional study conducted in Liège, Belgium, showed that clinically apparent pituitary adenomas were present in about 1 in 1000 inhabitants, which is 4 to 5 times the previously reported prevalence. Pituitary adenomas are generally sporadic, but some are associated with familial-isolated tumoral syndromes (mainly MEN1 and Carney complex). With the recent characterization of FIPA (Familial Isolated Pituitary Adenomas), familial pituitary adenomas are now thought to account for 5% to 8% of all pituitary tumors. New genetic mechanisms are being identified, improving our understanding of the complex manifestations and sometimes unpredictable outcome of pituitary adenomas.
Beckers
Albert
A
Endocrinologie, CHU de Liège, Université de Liège, Domaine universitaire du Sart-Tilman, 4000 Liège, Belgique. albert.beckers@chu.ulg.ac.be
Jaffrain-Rea
Marie-Lise
ML
Daly
Adrian E
AE
D000236:Adenoma / Q000453:epidemiology / Q000235:genetics* / Q000503:physiopathology; D054756:Cyclic AMP-Dependent Protein Kinase RIalpha Subunit / Q000235:genetics; D005260:Female; D018703:Founder Effect; D005602:France / Q000453:epidemiology; D006801:Humans; D047908:Intracellular Signaling Peptides and Proteins / Q000737:chemistry / Q000235:genetics / Q000502:physiology; D008297:Male; D018761:Multiple Endocrine Neoplasia Type 1 / Q000235:genetics; D009154:Mutation; D009363:Neoplasm Proteins / Q000235:genetics / Q000502:physiology; D009386:Neoplastic Syndromes, Hereditary / Q000453:epidemiology / Q000235:genetics*; D010911:Pituitary Neoplasms / Q000453:epidemiology / Q000235:genetics* / Q000503:physiopathology; D015995:Prevalence; D011518:Proto-Oncogene Proteins / Q000235:genetics / Q000502:physiology
D054756:Cyclic AMP-Dependent Protein Kinase RIalpha Subunit; D047908:Intracellular Signaling Peptides and Proteins; C105373:MEN1 protein, human; D009363:Neoplasm Proteins; D011518:Proto-Oncogene Proteins; C103265:aryl hydrocarbon receptor-interacting protein