ENETS Consensus Guidelines for the Management of Patients with Gastroduodenal Neoplasms

Abstract

ed in different studies classified as: duodenal carcinoid; 
\n duodenal gastroenteropancreatic (GEP) tumor; duodenal 
\npancreatic neuroendocrine tumor (p-NET); duodenal 
\ngastrinoma; duodenal somatostatinoma; gangliocytic 
\nparaganglioma; ampullary carcinoid or somatostatinoma; argentaffin carcinoid-producing serotonin of the 
\n duodenum; psammomatous somatostatinoma; duodenal 
\nNE carcinoma, poorly differentiated and small-cell NE 
\ncarcinoma of the duodenum [6] . The clinical and management aspect of duodenal gastrinomas are included in 
\nthe ‘Endocrine Tumors of the Pancreas – Gastrinoma’ 
\nsection and duodenal gastrinomas will only be considered in this section in comparison with the other
\nd-NENs.
\n Introduction 
\n Gastric neuroendocrine neoplasms (g-NENs) [1] are 
\nincreasingly recognized due to expanding indications of 
\nupper gastrointestinal (UGI) endoscopy. Often silent and 
\nbenign, g-NENs may, however, be aggressive when sporadic and may sometimes mimic the course of gastric adenocarcinoma.
\n Duodenal neuroendocrine tumors (d-NENs) [2] may 
\nor may not be associated with a functional clinical syndrome. The term d-NEN includes all duodenal tumors 
\nwith neuroendocrine (NE) features as determined by histological/immunohistochemical methods including positivity for NE cytosolic markers (neuron-specific enolase 
\n(NSE), PGP 9.5) or secretory vesicle proteins (chromogranin A (CgA), synaptophysin) and also frequently the 
\npresence of specific gastrointestinal (GI) hormones [3–8] . 
\nThe term d-NEN in this paper refers to tumors includ-
\n Published online: February 15, 2012 
\n Gianfranco Delle Fave 
\n Digestive and Liver Disease Unit 
\n ‘Sapienza’ University of Rome, Sant’Andrea Hospital 
\n Via Di Grottarossa 1035–1039, IT–00189 Rome (Italy) 
\n Tel. [phone], E-Mail gianfranco.dellefave @ uniroma1.it 
\n © 2012 S. Karger AG, Basel
\n0028–3835/12/0952–0074$38.00/0 
\n Accessible online at:
\nwww.karger.com/nen 
\n1