Neuroendocrine tumors of the small intestines are a rare disease with an incidence of 1 per 100,000 of the population; however, they account for over 20% of all neuroendocrine tumors and are the most common malignancy of the small intestines. Diagnosis is often made at a late stage of the disease, as the clinical symptoms are unspecific. Approximately one third of all patients show hepatic metastases at the time of diagnosis. There is no correlation between tumor size and tumor stage. Even small tumors <10 mm harbor the risk for lymphatic and distant metastases so that the extent of surgery is not determined by the size of the primary tumor. The intestinal-sparing resection with systematic lymphadenectomy along the superior mesenteric artery and removal of the retropancreatic lymph nodes is recommended even in localized stages of the disease.
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