PMID- 19522822 OWN - NLM STAT- MEDLINE DCOM- 20090713 LR - 20090615 IS - 1365-2796 (Electronic) IS - 0954-6820 (Linking) VI - 266 IP - 1 DP - 2009 Jul TI - Familial pituitary adenomas. PG - 5-18 LID - 10.1111/j.1365-2796.2009.02109.x [doi] AB - The majority of pituitary adenomas occur sporadically, however, about 5% of all cases occur in a familial setting, of which over half are due to multiple endocrine neoplasia type 1 (MEN-1) and Carney's complex (CNC). Since the late 1990s we have described non-MEN1/CNC familial pituitary tumours that include all tumour phenotypes, a condition named familial isolated pituitary adenomas (FIPA). The clinical characteristics of FIPA vary from those of sporadic pituitary adenomas, as patients with FIPA have a younger age at diagnosis and larger tumours. About 15% of FIPA patients have mutations in the aryl hydrocarbon receptor interacting protein gene (AIP), which indicates that FIPA may have a diverse genetic pathophysiology. This review describes the clinical features of familial pituitary adenomas like MEN1, the MEN 1-like syndrome MEN-4, CNC, FIPA, the tumour pathologies found in this setting and the genetic/molecular data that have been recently reported. FAU - Tichomirowa, M A AU - Tichomirowa MA AD - Department of Endocrinology, Centre Hospitalier Universitaire de Liege, University of Liege, Domaine Universitaire du Sart-Tilman, Liege 4000, Belgium. FAU - Daly, A F AU - Daly AF FAU - Beckers, A AU - Beckers A LA - eng PT - Journal Article PT - Review PL - England TA - J Intern Med JT - Journal of internal medicine JID - 8904841 SB - IM MH - Cell Transformation, Neoplastic/genetics MH - Female MH - Humans MH - Male MH - Multiple Endocrine Neoplasia Type 1/genetics MH - Mutation MH - Neoplastic Syndromes, Hereditary/*genetics MH - Pituitary Neoplasms/*genetics RF - 147 EDAT- 2009/06/16 09:00 MHDA- 2009/07/14 09:00 CRDT- 2009/06/16 09:00 PHST- 2009/06/16 09:00 [entrez] PHST- 2009/06/16 09:00 [pubmed] PHST- 2009/07/14 09:00 [medline] AID - JIM2109 [pii] AID - 10.1111/j.1365-2796.2009.02109.x [doi] PST - ppublish SO - J Intern Med. 2009 Jul;266(1):5-18. doi: 10.1111/j.1365-2796.2009.02109.x.