Pituitary adenomas can occur in a familial context, or they can be isolated 
cases, sometimes due to a predisposing syndrome. In multiple endocrine neoplasia 
type 1, they often associate with a mutation of the menin gene, a 
tumor-suppressing gene. A new germinal mutation predisposing to the development 
of multiple endocrine neoplasias has recently been identified in MENI-negative 
subjects on the gene CDKN1B encoding for p27(kip1)protein. Carney Complex 
syndrome--a rare disease--is in more than 60% of the cases linked to the 
inactivation mutation of a gene located on 17q22-24 that encodes the regulatory 
subunit 1 of protein kinase A, PRKARIA. Isolated familial pituitary adenomas 
represent 1.9 to 3.2% of the population of subjects presenting a pituitary 
adenoma. Low penetrance non-sense mutations, Q14X, IVS3-IG>A and R304X, in 
11q12-11q13 region encoding AIP protein, (Aryl hydrocarbon receptor Interacting 
Protein), have been described by Vierimaa et al, in Finish patients with 
pituitary adenoma predispositions.