PMID- 18310290
OWN - NLM
STAT- MEDLINE
DCOM- 20080508
LR  - 20080623
IS  - 1351-0088 (Print)
IS  - 1351-0088 (Linking)
VI  - 15
IP  - 1
DP  - 2008 Mar
TI  - Somatostatin-producing neuroendocrine tumors of the duodenum and pancreas: 
      incidence, types, biological behavior, association with inherited syndromes, and 
      functional activity.
PG  - 229-41
LID - 10.1677/ERC-07-0157 [doi]
AB  - Somatostatin-producing neuroendocrine tumors (SOM-NETs) of the duodenum and 
      pancreas appear to be heterogeneous. To determine their clinicopathological 
      profiles, respective data were analyzed on a series of 82 duodenal and 541 
      pancreatic NETs. In addition, the clinical records of 821 patients with duodenal 
      or pancreatic NETs were reviewed for evidence of a somatostatinoma syndrome. 
      Predominant or exclusive expression of somatostatin was found in 21 (26%) 
      duodenal and 21 (4%) pancreatic NETs. They were classified as sporadic (n=31) or 
      neurofibromatosis type 1 (NF1)-associated duodenal NETs (n=3), gangliocytic 
      paragangliomas (GCPGs; n=6), or poorly differentiated neuroendocrine carcinomas 
      (pdNECs; n=2). In addition, five duodenal and four pancreatic SOM-NETs were found 
      in five patients with multiple endocrine neoplasia type 1 (MEN1). Metastases 
      occurred in 13 (43%) patients with sporadic or NF1-associated SOM-NETs, but in 
      none of the duodenal or pancreatic MEN1-associated SOM-NETs or GCPGs. Sporadic 
      advanced (stage IV) SOM-NETs were more commonly detected in the pancreas than in 
      the duodenum. None of the patients (including the 821 patients for whom only the 
      clinical records were reviewed) fulfilled the criteria of a somatostatinoma 
      syndrome. Our data show that somatostatin expression is not only seen in sporadic 
      NETs but may also occur in GCPGs, pdNECs, and hereditary NETs. Surgical treatment 
      is effective in most duodenal and many pancreatic SOM-NETs. MEN1-associated 
      SOM-NETs and GCPGs follow a benign course, while somatostatin-producing pdNECs 
      are aggressive neoplasms. The occurrence of the so-called somatostatinoma 
      syndrome appears to be extremely uncommon.
FAU - Garbrecht, Nele
AU  - Garbrecht N
AD  - Department of Pathology, University of Kiel, Michaelisstr 11, Kiel, Germany. 
      ngarbrecht@path.uni-kiel.de
FAU - Anlauf, Martin
AU  - Anlauf M
FAU - Schmitt, Anja
AU  - Schmitt A
FAU - Henopp, Tobias
AU  - Henopp T
FAU - Sipos, Bence
AU  - Sipos B
FAU - Raffel, Andreas
AU  - Raffel A
FAU - Eisenberger, Claus F
AU  - Eisenberger CF
FAU - Knoefel, Wolfram T
AU  - Knoefel WT
FAU - Pavel, Marianne
AU  - Pavel M
FAU - Fottner, Christian
AU  - Fottner C
FAU - Musholt, Thomas J
AU  - Musholt TJ
FAU - Rinke, Anja
AU  - Rinke A
FAU - Arnold, Rudolf
AU  - Arnold R
FAU - Berndt, Uta
AU  - Berndt U
FAU - Plockinger, Ursula
AU  - Plockinger U
FAU - Wiedenmann, Bertram
AU  - Wiedenmann B
FAU - Moch, Holger
AU  - Moch H
FAU - Heitz, Philipp U
AU  - Heitz PU
FAU - Komminoth, Paul
AU  - Komminoth P
FAU - Perren, Aurel
AU  - Perren A
FAU - Kloppel, Gunter
AU  - Kloppel G
LA  - eng
PT  - Journal Article
PT  - Multicenter Study
PT  - Research Support, Non-U.S. Gov't
PL  - England
TA  - Endocr Relat Cancer
JT  - Endocrine-related cancer
JID - 9436481
RN  - 51110-01-1 (Somatostatin)
SB  - IM
MH  - Adult
MH  - Aged
MH  - Aged, 80 and over
MH  - Duodenal Neoplasms/metabolism/*pathology
MH  - Female
MH  - Follow-Up Studies
MH  - *Genetic Predisposition to Disease
MH  - Humans
MH  - Incidence
MH  - Male
MH  - Middle Aged
MH  - Multiple Endocrine Neoplasia Type 1/metabolism/pathology
MH  - Neuroendocrine Tumors/genetics/*metabolism/*pathology
MH  - Pancreatic Neoplasms/metabolism/*pathology
MH  - Paraganglioma/metabolism/pathology
MH  - Prognosis
MH  - Somatostatin/*metabolism
MH  - Somatostatinoma/metabolism/pathology
MH  - Syndrome
EDAT- 2008/03/04 09:00
MHDA- 2008/05/09 09:00
CRDT- 2008/03/04 09:00
PHST- 2008/03/04 09:00 [pubmed]
PHST- 2008/05/09 09:00 [medline]
PHST- 2008/03/04 09:00 [entrez]
AID - 15/1/229 [pii]
AID - 10.1677/ERC-07-0157 [doi]
PST - ppublish
SO  - Endocr Relat Cancer. 2008 Mar;15(1):229-41. doi: 10.1677/ERC-07-0157.