PMID- 17006979
OWN - NLM
STAT- MEDLINE
DCOM- 20061103
LR  - 20190430
IS  - 1007-9327 (Print)
IS  - 2219-2840 (Electronic)
IS  - 1007-9327 (Linking)
VI  - 12
IP  - 34
DP  - 2006 Sep 14
TI  - Sporadic versus hereditary gastrinomas of the duodenum and pancreas: distinct 
      clinico-pathological and epidemiological features.
PG  - 5440-6
AB  - Gastrinomas are defined as gastrin secreting tumors that are associated with 
      Zollinger-Ellison syndrome (ZES). ZES is characterized by elevated fasting 
      gastrin serum levels, positive secretin stimulation test and clinical symptoms 
      such as recurrent peptic ulcer disease, gastroesophageal reflux disease and 
      occasional diarrhea. Genetically, nonhereditary (sporadic) gastrinomas are 
      distinguished from hereditary gastrinomas, which are associated with multiple 
      endocrine neoplasia type 1 (MEN1) syndrome. In general, duodenal gastrinomas are 
      small and solitary if they are sporadic and multiple as well as hereditary. The 
      sporadic gastrinomas occur in the duodenum or in the pancreas while the 
      hereditary gastrinomas almost all occur in the duodenum. Our series of 77 
      sporadic duodenal neuroendocrine tumors (NETs) includes 18 patients (23.4%) with 
      gastrinomas and ZES. Of 535 sporadic NETs in the pancreas collected from the NET 
      archives of the departments of pathology in Zurich, Switzerland, and Kiel, 
      Germany, 24 patients (4.5%) suffered from sporadic pancreatic gastrinomas and 
      ZES. These NETs have to be distinguished from tumors with immunohistochemical 
      positivity for gastrin but without evidence of ZES. An additional 19 patients 
      suffered from MEN1 and ZES. These patients showed exclusively duodenal 
      gastrinomas, but not pancreatic gastrinomas. The prognosis of sporadic and 
      MEN1-associated duodenal gastrinomas is better than that of pancreatic 
      gastrinomas, since they progress slowly to liver metastasis. In summary, sporadic 
      and MEN1-associated gastrinomas in the duodenum and pancreas show different 
      clinico-pathological and genetic features. The incidence of sporadic duodenal 
      gastrin-producing tumors is increasing, possibly due to optimized diagnostic 
      procedures. In contrast, pancreatic MEN1-associated gastrinomas seem to be 
      extremely rare. A considerable subset of tumors with immunohistochemical 
      expression of gastrin but without evidence of ZES should be designated as 
      functionally inactive NETs expressing gastrin, but not as gastrinomas.
FAU - Anlauf, Martin
AU  - Anlauf M
AD  - Department of Pathology, University of Kiel, Michaelisstrasse 11, 24105 Kiel, 
      Germany. manlauf@path.uni-kiel.de
FAU - Garbrecht, Nele
AU  - Garbrecht N
FAU - Henopp, Tobias
AU  - Henopp T
FAU - Schmitt, Anja
AU  - Schmitt A
FAU - Schlenger, Regina
AU  - Schlenger R
FAU - Raffel, Andreas
AU  - Raffel A
FAU - Krausch, Markus
AU  - Krausch M
FAU - Gimm, Oliver
AU  - Gimm O
FAU - Eisenberger, Claus F
AU  - Eisenberger CF
FAU - Knoefel, Wolfram T
AU  - Knoefel WT
FAU - Dralle, Henning
AU  - Dralle H
FAU - Komminoth, Paul
AU  - Komminoth P
FAU - Heitz, Philipp U
AU  - Heitz PU
FAU - Perren, Aurel
AU  - Perren A
FAU - Kloppel, Gunter
AU  - Kloppel G
LA  - eng
PT  - Comparative Study
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PT  - Review
PL  - United States
TA  - World J Gastroenterol
JT  - World journal of gastroenterology
JID - 100883448
RN  - 0 (Gastrins)
SB  - IM
MH  - Duodenal Neoplasms/diagnosis/*epidemiology/genetics/*pathology
MH  - Gastrinoma/diagnosis/*epidemiology/genetics/*pathology
MH  - Gastrins/genetics/metabolism
MH  - Germany/epidemiology
MH  - Humans
MH  - Multiple Endocrine Neoplasia Type 1/diagnosis/epidemiology/genetics/pathology
MH  - Pancreatic Neoplasms/diagnosis/*epidemiology/genetics/*pathology
MH  - Prognosis
MH  - Switzerland/epidemiology
MH  - Zollinger-Ellison Syndrome/diagnosis/epidemiology/genetics/pathology
PMC - PMC4088224
EDAT- 2006/09/29 09:00
MHDA- 2006/11/04 09:00
PMCR- 2006/09/14
CRDT- 2006/09/29 09:00
PHST- 2006/09/29 09:00 [pubmed]
PHST- 2006/11/04 09:00 [medline]
PHST- 2006/09/29 09:00 [entrez]
PHST- 2006/09/14 00:00 [pmc-release]
AID - 10.3748/wjg.v12.i34.5440 [doi]
PST - ppublish
SO  - World J Gastroenterol. 2006 Sep 14;12(34):5440-6. doi: 10.3748/wjg.v12.i34.5440.