ENETS Consensus Guidelines for the management of patients with gastroduodenal neoplasms.

ed in different studies classified as: duodenal carcinoid; duodenal gastroenteropancreatic (GEP) tumor; duodenal pancreatic neuroendocrine tumor (p-NET); duodenal gastrinoma; duodenal somatostatinoma; gangliocytic paraganglioma; ampullary carcinoid or somatostatinoma; argentaffin carcinoid-producing serotonin of the duodenum; psammomatous somatostatinoma; duodenal NE carcinoma, poorly differentiated and small-cell NE carcinoma of the duodenum [6] . The clinical and management aspect of duodenal gastrinomas are included in the ‘Endocrine Tumors of the Pancreas – Gastrinoma’ section and duodenal gastrinomas will only be considered in this section in comparison with the other d-NENs. Introduction Gastric neuroendocrine neoplasms (g-NENs) [1] are increasingly recognized due to expanding indications of upper gastrointestinal (UGI) endoscopy. Often silent and benign, g-NENs may, however, be aggressive when sporadic and may sometimes mimic the course of gastric adenocarcinoma. Duodenal neuroendocrine tumors (d-NENs) [2] may or may not be associated with a functional clinical syndrome. The term d-NEN includes all duodenal tumors with neuroendocrine (NE) features as determined by histological/immunohistochemical methods including positivity for NE cytosolic markers (neuron-specific enolase (NSE), PGP 9.5) or secretory vesicle proteins (chromogranin A (CgA), synaptophysin) and also frequently the presence of specific gastrointestinal (GI) hormones [3–8] . The term d-NEN in this paper refers to tumors includ- Published online: February 15, 2012 Gianfranco Delle Fave Digestive and Liver Disease Unit ‘Sapienza’ University of Rome, Sant’Andrea Hospital Via Di Grottarossa 1035–1039, IT–00189 Rome (Italy) Tel. +39 06 3377 5691, E-Mail gianfranco.dellefave @ uniroma1.it © 2012 S. Karger AG, Basel 0028–3835/12/0952–0074$38.00/0 Accessible online at: www.karger.com/nen 1