PMID- 19407507 OWN - NLM STAT- MEDLINE DCOM- 20090723 LR - 20090501 IS - 1423-0046 (Electronic) IS - 0301-0163 (Linking) VI - 71 Suppl 2 DP - 2009 Apr TI - Genetic, molecular and clinical features of familial isolated pituitary adenomas. PG - 116-22 LID - 10.1159/000192448 [doi] AB - Pituitary adenomas occur in a familial setting in about 5% of all cases, and over half of these are due to multiple endocrine neoplasia type 1 (MEN1) and Carney's complex (CNC). Non-MEN1/CNC familial pituitary tumours of all tumour phenotypes, known as familial isolated pituitary adenomas (FIPA), were first described in the late 1990s. Clinical features of FIPA differ from those of sporadic pituitary adenomas, as patients with FIPA have a younger age at diagnosis and larger tumours. About 15% of patients with FIPA have mutations in the aryl hydrocarbon receptor-interacting protein gene (AIP), which indicates that FIPA may have a diverse genetic pathophysiology. This article describes the clinical features of FIPA, the tumour pathologies found in this setting and the genetic/molecular data that have recently been reported in FIPA. CI - Copyright 2009 S. Karger AG, Basel. FAU - Daly, Adrian F AU - Daly AF AD - Department of Endocrinology, Centre Hospitalier Universitaire de Liege, University of Liege, Liege, Belgium. FAU - Tichomirowa, Maria A AU - Tichomirowa MA FAU - Beckers, Albert AU - Beckers A LA - eng PT - Journal Article PT - Review DEP - 20090429 PL - Switzerland TA - Horm Res JT - Hormone research JID - 0366126 RN - 0 (Intracellular Signaling Peptides and Proteins) RN - 0 (MEN1 protein, human) RN - 0 (Proto-Oncogene Proteins) RN - 0 (aryl hydrocarbon receptor-interacting protein) SB - IM MH - Animals MH - *Genetic Diseases, Inborn/genetics/metabolism/pathology/physiopathology MH - Humans MH - Intracellular Signaling Peptides and Proteins/genetics/metabolism MH - *Pituitary Neoplasms/genetics/metabolism/pathology/physiopathology MH - Proto-Oncogene Proteins/genetics/metabolism RF - 43 EDAT- 2009/05/07 09:00 MHDA- 2009/07/25 09:00 CRDT- 2009/05/02 09:00 PHST- 2009/05/02 09:00 [entrez] PHST- 2009/05/07 09:00 [pubmed] PHST- 2009/07/25 09:00 [medline] AID - 000192448 [pii] AID - 10.1159/000192448 [doi] PST - ppublish SO - Horm Res. 2009 Apr;71 Suppl 2:116-22. doi: 10.1159/000192448. Epub 2009 Apr 29.