| Somatostatin-producing neuroendocrine tumors of the duodenum and pancreas: incidence, types, biological behavior, association with inherited syndromes, and functional activity. | |
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MedLine Citation:
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PMID: 18310290 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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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. |
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Authors:
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Nele Garbrecht; Martin Anlauf; Anja Schmitt; Tobias Henopp; Bence Sipos; Andreas Raffel; Claus F Eisenberger; Wolfram T Knoefel; Marianne Pavel; Christian Fottner; Thomas J Musholt; Anja Rinke; Rudolf Arnold; Uta Berndt; Ursula Plöckinger; Bertram Wiedenmann; Holger Moch; Philipp U Heitz; Paul Komminoth; Aurel Perren; Günter Klöppel |
Publication Detail:
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Type: Journal Article; Multicenter Study; Research Support, Non-U.S. Gov't |
Journal Detail:
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Title: Endocrine-related cancer Volume: 15 ISSN: 1351-0088 ISO Abbreviation: Endocr. Relat. Cancer Publication Date: 2008 Mar |
Date Detail:
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Created Date: 2008-03-03 Completed Date: 2008-05-08 Revised Date: 2008-06-23 |
Medline Journal Info:
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Nlm Unique ID: 9436481 Medline TA: Endocr Relat Cancer Country: England |
Other Details:
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Languages: eng Pagination: 229-41 Citation Subset: IM |
Affiliation:
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Department of Pathology, University of Kiel, Michaelisstr 11, Kiel, Germany. ngarbrecht@path.uni-kiel.de |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adult Aged Aged, 80 and over Duodenal Neoplasms / metabolism, pathology* Female Follow-Up Studies Genetic Predisposition to Disease* Humans Incidence Male Middle Aged Multiple Endocrine Neoplasia Type 1 / metabolism, pathology Neuroendocrine Tumors / genetics, metabolism*, pathology* Pancreatic Neoplasms / metabolism, pathology* Paraganglioma / metabolism, pathology Prognosis Somatostatin / metabolism* Somatostatinoma / metabolism, pathology Syndrome |
| Chemical | |
Reg. No./Substance:
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51110-01-1/Somatostatin |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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