PMID- 16699310
OWN - NLM
STAT- MEDLINE
DCOM- 20060616
LR  - 20071115
IS  - 0147-5185 (Print)
IS  - 0147-5185 (Linking)
VI  - 30
IP  - 5
DP  - 2006 May
TI  - Microadenomatosis of the endocrine pancreas in patients with and without the 
      multiple endocrine neoplasia type 1 syndrome.
PG  - 560-74
AB  - It has been suggested that microadenomatosis of the endocrine pancreas is a 
      hallmark of the multiple endocrine neoplasia type 1 syndrome (MEN1). This study 
      attempts to elucidate the relationship between pancreatic microadenomatosis and 
      the MEN1 and von Hippel-Lindau (VHL) syndromes. Pancreatic tissue specimens from 
      37 patients (with either microadenomatosis or the MEN1 syndrome) were analyzed 
      using immunohistochemistry, confocal laser scanning microscopy, and morphometric 
      methods. The MEN1 and the VHL status were assessed on the basis of clinical 
      criteria (all patients) and PCR-based mutational analysis (15 and 5 patients, 
      respectively). Pancreatic microadenomatosis was found in 35 of 37 patients, 28 of 
      whom fulfilled the clinicopathologic criteria and 13 the genetic criteria for 
      MEN1, whereas none of the patients had evidence of a VHL syndrome. Microadenomas 
      were present in 26 of the 28 MEN1 patients, and all these tumors were 
      consistently multihormonal. Five of the 9 patients with microadenomatosis and no 
      clinical evidence for MEN1 or VHL also lacked mutations for the respective genes. 
      Five of these 9 patients suffered from hyperinsulinism and revealed multiple 
      insulin-positive tumors. The other patients were nonsymptomatic and showed 
      multiple glucagon-expressing neoplasms. In microadenomatosis patients with and 
      without the MEN1 syndrome, a subset of morphologically normal-appearing islets 
      showed increased endocrine cell proliferation. In conclusion, endocrine 
      multihormonal microadenomatosis of the pancreas is a feature of MEN1. In 
      addition, a monohormonal type of pancreatic microadenomatosis was identified that 
      consisted of either insulinomas or glucagon-producing tumors and was not 
      associated with MEN1 or VHL.
FAU - Anlauf, Martin
AU  - Anlauf M
AD  - Departments of Pathology, University of Kiel, Michaelisstrasse 11, 24105 Kiel, 
      Germany. manlauf@path.uni-kiel.de
FAU - Schlenger, Regina
AU  - Schlenger R
FAU - Perren, Aurel
AU  - Perren A
FAU - Bauersfeld, Juliane
AU  - Bauersfeld J
FAU - Koch, Christian A
AU  - Koch CA
FAU - Dralle, Henning
AU  - Dralle H
FAU - Raffel, Andreas
AU  - Raffel A
FAU - Knoefel, Wolfram T
AU  - Knoefel WT
FAU - Weihe, Eberhard
AU  - Weihe E
FAU - Ruszniewski, Philippe
AU  - Ruszniewski P
FAU - Couvelard, Anne
AU  - Couvelard A
FAU - Komminoth, Paul
AU  - Komminoth P
FAU - Heitz, Philipp U
AU  - Heitz PU
FAU - Kloppel, Gunter
AU  - Kloppel G
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - United States
TA  - Am J Surg Pathol
JT  - The American journal of surgical pathology
JID - 7707904
SB  - IM
CIN - Am J Surg Pathol. 2006 Dec;30(12):1630; author reply 1630-1. PMID: 17122523
MH  - Adenoma, Islet Cell/*complications/*pathology
MH  - Adult
MH  - Aged
MH  - DNA Mutational Analysis
MH  - Female
MH  - Humans
MH  - Immunohistochemistry
MH  - Male
MH  - Microscopy, Confocal
MH  - Middle Aged
MH  - Multiple Endocrine Neoplasia Type 1/*complications/genetics/*pathology
MH  - Mutation
MH  - Pancreatic Neoplasms/*complications/*pathology
MH  - Polymerase Chain Reaction
MH  - von Hippel-Lindau Disease/complications/genetics/pathology
EDAT- 2006/05/16 09:00
MHDA- 2006/06/17 09:00
CRDT- 2006/05/16 09:00
PHST- 2006/05/16 09:00 [pubmed]
PHST- 2006/06/17 09:00 [medline]
PHST- 2006/05/16 09:00 [entrez]
AID - 00000478-200605000-00002 [pii]
AID - 10.1097/01.pas.0000194044.01104.25 [doi]
PST - ppublish
SO  - Am J Surg Pathol. 2006 May;30(5):560-74. doi: 10.1097/01.pas.0000194044.01104.25.