PMID- 19170361
OWN - NLM
STAT- MEDLINE
DCOM- 20090224
LR  - 20160307
IS  - 1784-3286 (Print)
IS  - 1784-3286 (Linking)
VI  - 63
IP  - 6
DP  - 2008 Nov-Dec
TI  - Update on the treatment of pituitary adenomas: familial and genetic 
      considerations.
PG  - 418-24
AB  - Clinically-relevant pituitary adenomas occur with a prevalence of approximately 1 
      per 1000 population in Belgium. Pituitary adenomas that occur in families are 
      likely to have an important genetic pathophysiological basis. Currently about 5% 
      of all pituitary adenoma cases have a family history of pituitary adenomas, 
      classically due to multiple endocrine neoplasia type 1 (MEN1) and Carney complex 
      (CNC). Over the last decade we have described non-MEN1/CNC familial pituitary 
      tumours that include all tumour phenotypes, a condition named '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 tumours at diagnosis. Approximately 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. In this review we examine 
      new findings on the epidemiology of pituitary adenomas and we review familial 
      causes of pituitary adenomas with a particular emphasis on modern clinical 
      testing. In addition, the clinical and genetic features of FIPA are described as 
      FIPA represents a useful framework to study the features of pituitary adenomas 
      that occur in a familial setting.
FAU - Daly, A F
AU  - Daly AF
AD  - Department of Endocrinology, Centre Hospitalier Universitaire de Liege, 
      University of Liege, Liege, Belgium. adrian.daly@ulg.ac.be
FAU - Beckers, A
AU  - Beckers A
LA  - eng
PT  - Journal Article
PT  - Review
PL  - England
TA  - Acta Clin Belg
JT  - Acta clinica Belgica
JID - 0370306
RN  - 0 (Intracellular Signaling Peptides and Proteins)
RN  - 0 (aryl hydrocarbon receptor-interacting protein)
SB  - IM
MH  - Acromegaly
MH  - Adenoma/*therapy
MH  - Animals
MH  - Humans
MH  - Intracellular Signaling Peptides and Proteins/genetics
MH  - Loss of Heterozygosity
MH  - Multiple Endocrine Neoplasia Type 1/complications/genetics
MH  - Mutation
MH  - Pituitary Neoplasms/*drug therapy/etiology/*genetics
MH  - Prolactinoma/drug therapy/genetics
RF  - 54
EDAT- 2009/01/28 09:00
MHDA- 2009/02/25 09:00
CRDT- 2009/01/28 09:00
PHST- 2009/01/28 09:00 [entrez]
PHST- 2009/01/28 09:00 [pubmed]
PHST- 2009/02/25 09:00 [medline]
AID - 10.1179/acb.2008.086 [doi]
PST - ppublish
SO  - Acta Clin Belg. 2008 Nov-Dec;63(6):418-24. doi: 10.1179/acb.2008.086.