PMID- 16509219
OWN - NLM
STAT- MEDLINE
DCOM- 20060406
LR  - 20091021
IS  - 0030-6002 (Print)
IS  - 0030-6002 (Linking)
VI  - 147
IP  - 5
DP  - 2006 Feb 5
TI  - [Hereditary tumours of the endocrine pancreas].
PG  - 195-200
AB  - The pathogenesis, diagnosis and therapy of tumours originating from the endocrine 
      pancreas represent one of the most exciting challenges of contemporary medicine. 
      Some of these tumours appear as part of four hereditary syndromes (multiple 
      endocrine neoplasia type 1 (MEN1), von Hippel-Lindau disease (VHL), 
      neurofibromatosis type 1 and tuberous sclerosis) that are all inherited as 
      autosomal dominant traits and result from mutations of tumour suppressor genes. 
      Considering its clinical relevance, MEN1 appears to be the most important among 
      these four syndromes. Tumours of the endocrine pancreas develop in 30-80% of 
      patients carrying mutations of the MEN1 gene. Gastrinomas are the most frequent 
      functioning tumours in MEN1 patients, followed by insulinomas, whereas other 
      tumors e.g. glucagonoma, VIP-oma, GRF-oma and somatostatinoma occur very rarely. 
      Tumours of the endocrine pancreas are infrequent in patients suffering from VHL, 
      neurofibromatosis and tuberous sclerosis. In this review article, the authors 
      present a synopsis of tumours of the endocrine pancreas related to these 
      hereditary syndromes underlining the clinical characteristics, diagnostical and 
      therapeutical possibilities.
FAU - Igaz, Peter
AU  - Igaz P
AD  - Semmelweis Egyetem, Altalanos Orvostudomanyi Kar, II. Belgyogyaszati Klinika. 
      igapet@bel2.sote.hu
FAU - Igaz, Ivan
AU  - Igaz I
FAU - Racz, Karoly
AU  - Racz K
FAU - Tulassay, Zsolt
AU  - Tulassay Z
LA  - hun
PT  - English Abstract
PT  - Journal Article
PT  - Review
TT  - Az endokrin pancreas oroklodo daganatai.
PL  - Hungary
TA  - Orv Hetil
JT  - Orvosi hetilap
JID - 0376412
SB  - IM
MH  - Gastrinoma/diagnosis/genetics
MH  - Glucagonoma/genetics
MH  - Humans
MH  - Insulinoma/diagnosis/genetics
MH  - Mass Screening
MH  - Multiple Endocrine Neoplasia Type 1/complications
MH  - Neurofibromatosis 1/complications
MH  - Pancreatic Neoplasms/*diagnosis/*genetics/prevention & control
MH  - Somatostatinoma/genetics
MH  - Tuberous Sclerosis/complications
MH  - Vipoma/genetics
MH  - von Hippel-Lindau Disease/complications
RF  - 50
EDAT- 2006/03/03 09:00
MHDA- 2006/04/07 09:00
CRDT- 2006/03/03 09:00
PHST- 2006/03/03 09:00 [pubmed]
PHST- 2006/04/07 09:00 [medline]
PHST- 2006/03/03 09:00 [entrez]
PST - ppublish
SO  - Orv Hetil. 2006 Feb 5;147(5):195-200.