[Clinical and genetic characterization of FIPA (familial isolated pituitary adenomas)].
Pituitary adenomas are common brain tumours at autopsy and radiological series of unselected population. Historically, few epidemiologic data regarding the prevalence of clinically apparent pituitary adenomas have been available. Recently, a cross-sectional study conducted in Liège, Belgium, noted that clinically-apparent pituitary adenomas occurred with a prevalence of 1:1064 inhabitants, which is 3.5-5 times the previously reported prevalence. Pituitary adenomas occur predominantly as sporadic tumors, but also in a familial setting or associated to some familial/isolated tumoral syndromes. The recent characterization of the novel clinical entity FIPA (Familial Isolated Pituitary Adenomas) increased the prevalence of familial pituitary adenomas which account now for about 5% of pituitary tumors. Distinct genetic mechanisms are continuously identified and increase our understanding of the complex clinical presentation and sometimes unpredictable evolution of pituitary adenomas.
Beckers
A
A
Service d'Endocrinologie, CHU de Liège, Liège, Belgique.
Apetrii
P
P
Daly
A
A
Tichomirova
M
M
Vanbellingen
J F
JF
Georges
M
M
Bours
V
V
D000172:Acromegaly / Q000209:etiology; D000236:Adenoma / Q000175:diagnosis / Q000235:genetics*; D056733:Carney Complex / Q000235:genetics; D005359:Fibrous Dysplasia, Polyostotic / Q000235:genetics; D006801:Humans; D009377:Multiple Endocrine Neoplasia / Q000235:genetics; D010911:Pituitary Neoplasms / Q000175:diagnosis / Q000235:genetics*