Abstract
Neuroendocrine neoplasias (NEN) of the stomach, duodenum, pancreas, appendix, or rectum that are ≤1 cm in size as well as well-differentiated with World Health Organization grade 1 (G1) can be considered 'early' neuroendocrine tumors; they have a very good prognosis. Regarding prognosis, neuroendocrine tumors (NET) G1 must be distinguished from well-differentiated NET G2 and poorly differentiated neuroendocrine carcinomas (NEC) G3. NET are increasing, with a rise in the age-adjusted incidence in the USA by about 700% in the last 40 years. Earlier diagnosis of NET is one of the main epidemiological changes of clinically detected NEN. The general availability of high-resolution endoscopy and advanced radiological imaging techniques has contributed to a shift in the discovery to smaller-sized (≤10 mm) gastrointestinal and pancreatic NET and earlier tumor stages at diagnosis. Thus, screening colonoscopy is effective in the early diagnosis not only of colorectal adenomas and adenocarcinomas but also of rectal NET. Endoscopic resection is the treatment of choice in NET G1 of the stomach, duodenum (despite gastrinoma), and rectum that are ≤10 mm in size, do not infiltrate the muscularis propria (T1), and do not show angioinvasion (V0, L0). Similarly, histologically proven, early pancreatic NET G1 (≤10 mm) may be managed conservatively by regular surveillance. In contrast, small (≤1 cm) NET G1 of the jejunum or ileum are not 'early' tumors and have to be resected surgically with lymph node dissection.