PMID- 17179192
OWN - NLM
STAT- MEDLINE
DCOM- 20070510
LR  - 20220309
IS  - 0021-972X (Print)
IS  - 0021-972X (Linking)
VI  - 92
IP  - 3
DP  - 2007 Mar
TI  - Multiple endocrine neoplasia type 1 (MEN1): loss of one MEN1 allele in tumors and 
      monohormonal endocrine cell clusters but not in islet hyperplasia of the 
      pancreas.
PG  - 1118-28
AB  - CONTEXT: The occurrence of multiple small pancreatic endocrine tumors in patients 
      suffering from multiple endocrine neoplasia type 1 (MEN1) represents a unique 
      possibility to study early neoplasms and their potential precursor lesions. To 
      date, it is unknown whether small islet-like endocrine cell clusters found in 
      MEN1 patients are neoplastic or rather hyperplastic. It is also unclear whether 
      microadenomas develop from islets. DESIGN: We hypothesized that monohormonal 
      endocrine cell clusters observed in MEN1 patients are small neoplasms with loss 
      of heterozygosity of the MEN1 locus. Using a technique combining fluorescence in 
      situ hybridization of the MEN1 locus and the centromeric region of chromosome 11q 
      with hormone immunostaining, we examined resection specimens from four MEN1 
      patients. We focused our investigations on the following: 1) typical 
      microadenomas; 2) monohormonal endocrine cell clusters; 3) endocrine and exocrine 
      structures entrapped in microadenomas; and 4) morphologically normal islets. 
      RESULTS: Loss of one MEN1 allele was found in all 27 microadenomas and 19 of 20 
      (95%) monohormonal endocrine cell clusters. By contrast, it was absent in islets 
      and ductal or acinar structures. Our results indicate that monohormonal endocrine 
      cell clusters represent a minute form of microadenomas. CONCLUSION: The frequent 
      presence of single nonneoplastic insulin cells in microadenomas and the 
      occurrence of microadenomas in islets suggest an islet origin of microadenomas. 
      Islet hyperplasia does not seem to be an obligatory stage in pancreatic 
      MEN1-associated tumor development.
FAU - Perren, Aurel
AU  - Perren A
AD  - Institute of Surgical Pathology, Department of Pathology, University Hospital 
      Zurich, 8091 Zurich, Switzerland. aurel.perren@usz.ch
FAU - Anlauf, Martin
AU  - Anlauf M
FAU - Henopp, Tobias
AU  - Henopp T
FAU - Rudolph, Thomas
AU  - Rudolph T
FAU - Schmitt, Anja
AU  - Schmitt A
FAU - Raffel, Andreas
AU  - Raffel A
FAU - Gimm, Oliver
AU  - Gimm O
FAU - Weihe, Eberhard
AU  - Weihe E
FAU - Knoefel, Wolfram T
AU  - Knoefel WT
FAU - Dralle, Henning
AU  - Dralle H
FAU - Heitz, Philipp U
AU  - Heitz PU
FAU - Komminoth, Paul
AU  - Komminoth P
FAU - Kloppel, Gunter
AU  - Kloppel G
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
DEP - 20061219
PL  - United States
TA  - J Clin Endocrinol Metab
JT  - The Journal of clinical endocrinology and metabolism
JID - 0375362
RN  - 0 (MEN1 protein, human)
RN  - 0 (Proto-Oncogene Proteins)
SB  - IM
CIN - J Clin Endocrinol Metab. 2007 Mar;92(3):811-2. PMID: 17341576
MH  - Adult
MH  - Aged
MH  - Carcinoma, Pancreatic Ductal/*genetics/pathology
MH  - Female
MH  - Humans
MH  - Hyperplasia/genetics
MH  - *Loss of Heterozygosity
MH  - Male
MH  - Middle Aged
MH  - Models, Biological
MH  - Multiple Endocrine Neoplasia Type 1/*genetics/pathology
MH  - Pancreas/*pathology
MH  - Pancreatic Neoplasms/*genetics/pathology
MH  - Proto-Oncogene Proteins/*genetics
EDAT- 2006/12/21 09:00
MHDA- 2007/05/11 09:00
CRDT- 2006/12/21 09:00
PHST- 2006/12/21 09:00 [pubmed]
PHST- 2007/05/11 09:00 [medline]
PHST- 2006/12/21 09:00 [entrez]
AID - jc.2006-1944 [pii]
AID - 10.1210/jc.2006-1944 [doi]
PST - ppublish
SO  - J Clin Endocrinol Metab. 2007 Mar;92(3):1118-28. doi: 10.1210/jc.2006-1944. Epub 
      2006 Dec 19.