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Department of Pathology, University of Kiel, Michaelisstr. 11, 24105 Kiel, Germany¹ Department of Pathology, University Hospital of Zürich, 8091 Zürich, Switzerland² Department of General, Visceral and Pediatric Surgery, University of Düsseldorf, 40228 Düsseldorf, Germany³ Department of Gastroenterology and Endocrinology, University of Erlangen, 91054 Erlangen, Germany⁴ Department of Endocrinology, University of Mainz, 55131 Mainz, Germany⁵ Department of General and Abdominal Surgery, University of Mainz, 55131 Mainz, Germany⁶ Department of Gastroenterology and Endocrinology, University of Marburg, 35033 Marburg, Germany⁷ Department of Hepatology and Gastroenterology, Charité, Campus Virchow Klinikum, 13353 Berlin, Germany⁸ Department of Pathology, Triemli Spital, 8063 Zürich, Switzerland⁹ Institute of Pathology, Technical University, 8165 Munich, Germany

(Correspondence should be addressed to N Garbrecht; Email: ngarbrecht{at}path.uni-kiel.de)

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.