Neuroendocrine tumors (NETs) of the small bowels are on the rise: in the US they have increased by 300-500% in the last 35 years. At the same time their prognosis has been much improved. Most NETs of the duodenum are nowadays detected "incidentally" and therefore recognized at an early stage. Duodenal NETs that are well differentiated, not larger than 10 mm in greatest dimension and limited to the mucosa/submucosa can be endoscopically resected. In NETs with a size between 10 mm and 20 mm the therapeutic strategy has to be individually discussed. Endoscopic ultrasound is the method of choice to determine tumor size and depth of infiltration. Surgery is indicated for well differentiated duodenal NETs greater than 20 mm, for localized sporadic gastrinomas and for localized poorly differentiated NE cancers. Surgery is also indicated for localized/regional ileal NETs. Advanced ileal NETs with a carcinoid syndrome are treated with stable somatostatin analogs. This treatment also significantly improves the (progression-free) survival in patients with metastatic NETs of the ileum. For optimal NET management tumor biology, type, localization and stage of the neoplasm as well as the individual situation of the patient have to be taken into account.
Georg Thieme Verlag KG Stuttgart, New York.