PMID- 17890894
OWN - NLM
STAT- MEDLINE
DCOM- 20071227
LR  - 20070924
IS  - 1423-0291 (Electronic)
IS  - 1015-2008 (Linking)
VI  - 74
IP  - 5
DP  - 2007
TI  - Endocrine precursor lesions and microadenomas of the duodenum and pancreas with 
      and without MEN1: criteria, molecular concepts and clinical significance.
PG  - 279-84
AB  - Proliferative changes in the neuroendocrine cells that precede neoplasia are of 
      interest for the understanding of tumorigenesis and the early recognition of 
      neuroendocrine tumors. This review focuses on precursor lesions of duodenal and 
      pancreatic neuroendocrine tumors in multiple endocrine neoplasia type 1 (MEN1) 
      and also discusses 2 new disease entities of pancreatic microadenomatosis. The 
      gastrinomas observed in MEN1 are almost exclusively localized in the duodenum and 
      are multicentric. It has been shown that, in contrast to sporadic duodenal 
      gastrinomas, they are associated with hyperplastic gastrin cell lesions and tiny 
      gastrin-producing microtumors less than 500 microm in diameter. In the pancreas, 
      microadenomatosis (multiple tumors up to 5 mm in diameter) is a feature of MEN1. 
      These microadenomas predominantly express glucagon and pancreatic polypeptide, 
      but do not cause a hormonal syndrome. Approximately 50% of MEN1 minigastrinomas 
      in the duodenum and almost all microadenomas in the pancreas show allelic 
      deletion of the MEN1 gene and therefore may represent 'initial' neoplasms. In 
      contrast, endocrine cell precursor lesions retain heterozygosity. Pancreatic 
      microadenomatosis was also found unassociated with hereditary syndromes and 2 
      monohormonal types were identified: (1) glucagon-producing microadenomatosis and 
      (2) insulin-producing microadenomatosis, both associated with macrotumors. 
      Whether these types of microadenomatosis represent novel disease entities and how 
      to diagnose and treat these patients remains to be clarified by further studies.
CI  - Copyright 2007 S. Karger AG, Basel.
FAU - Anlauf, Martin
AU  - Anlauf M
AD  - Department of Pathology, University of Kiel, Kiel, Germany. 
      manlauf@path.uni-kiel.de
FAU - Perren, Aurel
AU  - Perren A
FAU - Kloppel, Gunter
AU  - Kloppel G
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PT  - Review
PL  - Switzerland
TA  - Pathobiology
JT  - Pathobiology : journal of immunopathology, molecular and cellular biology
JID - 9007504
SB  - IM
CIN - Pathobiology. 2007;74(5):277-8. PMID: 17890893
MH  - Adenoma/classification/genetics/*pathology
MH  - Carcinoma, Neuroendocrine/classification/genetics/pathology
MH  - Duodenal Neoplasms/classification/genetics/*pathology
MH  - Gastrinoma/classification/genetics/pathology
MH  - Humans
MH  - Multiple Endocrine Neoplasia Type 1/*genetics/*pathology
MH  - Pancreatic Neoplasms/classification/genetics/*pathology
MH  - Precancerous Conditions/classification/genetics/*pathology
RF  - 24
EDAT- 2007/09/25 09:00
MHDA- 2007/12/28 09:00
CRDT- 2007/09/25 09:00
PHST- 2006/11/22 00:00 [received]
PHST- 2007/01/30 00:00 [accepted]
PHST- 2007/09/25 09:00 [pubmed]
PHST- 2007/12/28 09:00 [medline]
PHST- 2007/09/25 09:00 [entrez]
AID - 000105810 [pii]
AID - 10.1159/000105810 [doi]
PST - ppublish
SO  - Pathobiology. 2007;74(5):279-84. doi: 10.1159/000105810.