Gastric neuroendocrine neoplasms (g-NENs) 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) 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.
The term d-NEN in this paper refers to tumors included 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. 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.
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