In addition, we summarize the clinical features of IFS families with AIP mutations: The adenomas are diagnosed at a young age and are larger than in IFS patients without AIP mutations or in sporadic somatotropinomas, indicating more aggressive disease.
Recently, germline mutations of the aryl hydrocarbon receptor-interacting protein gene (AIP) have been found in families with pituitary adenoma predisposition, FIPA, and IFS.
A minority of FIPA families overall (15%) exhibit mutations in the aryl hydrocarbon receptor-interacting protein (AIP) gene; AIP mutations are present in only half of IFS kindreds occurring as part of the FIPA cohort.
Recently, some cases of familial isolated somatotropinoma have been associated with germline mutations in the aryl hydrocarbon receptor interacting protein (AIP) gene.