PMID- 19153518
OWN - NLM
STAT- MEDLINE
DCOM- 20090416
LR  - 20130520
IS  - 1423-0046 (Electronic)
IS  - 0301-0163 (Linking)
VI  - 71 Suppl 1
DP  - 2009 Jan
TI  - Update on familial pituitary tumors: from multiple endocrine neoplasia type 1 to 
      familial isolated pituitary adenoma.
PG  - 105-11
LID - 10.1159/000178050 [doi]
AB  - BACKGROUND: 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 complex (CNC). Since the late 1990s, we have described 
      non-MEN1/CNC familial pituitary tumors that include all tumor phenotypes and have 
      named this condition 'familial isolated pituitary adenoma' (FIPA). Clinical 
      features of FIPA differ from those of sporadic pituitary adenomas in that 
      patients with FIPA are often younger and have larger tumors at diagnosis. 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. We review the clinical features of FIPA, the tumor pathologies 
      found in this setting and the genetic/molecular data that have been recently 
      reported. CONCLUSIONS: Clinically relevant pituitary adenomas are more common 
      than previously thought and occur in a familial setting in about 5% of cases 
      overall. Therefore, specific questioning regarding family history of pituitary 
      disease should be part of the workup of all patients with pituitary adenomas, not 
      just those with acromegaly. FIPA is a useful clinical framework to study the 
      features of pituitary adenomas that occur in a familial setting since it 
      encompasses all tumor phenotypes and heterogeneous/homogeneous expression among 
      affected family members.
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 - 20090121
PL  - Switzerland
TA  - Horm Res
JT  - Hormone research
JID - 0366126
RN  - 0 (Intracellular Signaling Peptides and Proteins)
RN  - 0 (aryl hydrocarbon receptor-interacting protein)
SB  - IM
EIN - Horm Res. 2009;71(5):297. Tichomirow, Maria A [corrected to Tichomirowa, Maria A]
MH  - Adenoma/*etiology/genetics
MH  - Humans
MH  - Intracellular Signaling Peptides and Proteins/genetics
MH  - Models, Biological
MH  - Multiple Endocrine Neoplasia Type 1/*complications/genetics
MH  - Mutation/physiology
MH  - Pituitary Neoplasms/*etiology/genetics
RF  - 38
EDAT- 2009/01/30 09:00
MHDA- 2009/04/17 09:00
CRDT- 2009/01/21 09:00
PHST- 2009/01/21 09:00 [entrez]
PHST- 2009/01/30 09:00 [pubmed]
PHST- 2009/04/17 09:00 [medline]
AID - 000178050 [pii]
AID - 10.1159/000178050 [doi]
PST - ppublish
SO  - Horm Res. 2009 Jan;71 Suppl 1:105-11. doi: 10.1159/000178050. Epub 2009 Jan 21.