PMID- 20961530
OWN - NLM
STAT- MEDLINE
DCOM- 20110401
LR  - 20161031
IS  - 2213-3941 (Electronic)
IS  - 0003-4266 (Linking)
VI  - 71
IP  - 6
DP  - 2010 Dec
TI  - Familial pituitary adenomas.
PG  - 479-85
LID - 10.1016/j.ando.2010.08.005 [doi]
AB  - Pituitary adenomas are benign intracranial neoplasms that present a major 
      clinical concern because of hormonal overproduction or compression symptoms of 
      adjacent structures. Most arise in a sporadic setting with a small percentage 
      developing as a part of familial syndromes such as multiple endocrine neoplasia 
      type 1 (MEN1), Carney complex (CNC), and the recently described familial isolated 
      pituitary adenomas (FIPA) and MEN-4. While the genetic alterations responsible 
      for the formation of sporadic adenomas remain largely unknown, considerable 
      advances have been made in defining culprit genes in these familial syndromes. 
      Mutations in MEN1 and PRKAR1A genes are found in the majority of MEN1 and CNC 
      patients, respectively. About 15% of FIPA kindreds present with mutations of the 
      aryl hydrocarbon receptor-interacting protein (AIP) gene. Mutations in the CDKN1B 
      gene, encoding p27(Kip)(1) were identified in MEN4 cases. Familial tumours appear 
      to differ from their sporadic counterparts not only in genetic basis but also in 
      clinical characteristics. Evidence suggests that, especially in MEN1 and FIPA, 
      they are more aggressive and affect patients at younger age, therefore justifying 
      the importance of early diagnosis. In this review, we summarize the genetic and 
      clinical characteristics of these familial pituitary adenomas.
CI  - Copyright (c) 2010. Published by Elsevier Masson SAS.
FAU - Vandeva, S
AU  - Vandeva S
AD  - Department of Endocrinology, University of Liege, CHU de Liege, Belgium.
FAU - Vasilev, V
AU  - Vasilev V
FAU - Vroonen, L
AU  - Vroonen L
FAU - Naves, L
AU  - Naves L
FAU - Jaffrain-Rea, M-L
AU  - Jaffrain-Rea ML
FAU - Daly, A F
AU  - Daly AF
FAU - Zacharieva, S
AU  - Zacharieva S
FAU - Beckers, A
AU  - Beckers A
LA  - eng
PT  - Journal Article
PT  - Review
DEP - 20101018
PL  - France
TA  - Ann Endocrinol (Paris)
JT  - Annales d'endocrinologie
JID - 0116744
SB  - IM
MH  - Adenoma/complications/*genetics
MH  - Carney Complex/etiology/genetics
MH  - Humans
MH  - Multiple Endocrine Neoplasia Type 1/etiology/genetics
MH  - Pituitary Neoplasms/complications/*genetics
EDAT- 2010/10/22 06:00
MHDA- 2011/04/02 06:00
CRDT- 2010/10/22 06:00
PHST- 2010/08/01 00:00 [received]
PHST- 2010/08/06 00:00 [revised]
PHST- 2010/08/06 00:00 [accepted]
PHST- 2010/10/22 06:00 [entrez]
PHST- 2010/10/22 06:00 [pubmed]
PHST- 2011/04/02 06:00 [medline]
AID - S0003-4266(10)00159-9 [pii]
AID - 10.1016/j.ando.2010.08.005 [doi]
PST - ppublish
SO  - Ann Endocrinol (Paris). 2010 Dec;71(6):479-85. doi: 10.1016/j.ando.2010.08.005. 
      Epub 2010 Oct 18.