Appendiceal neuroendocrine tumors are generally detected after appendectomy. Carcinoid syndrome generally occurs when jejuno-ileal neuroendocrine tumors metastasize to the liver. Duodenal and jejuno-ileal neuroendocrine tumors are distinct biologically and clinically. Patients with small intestinal neuroendrocrine tumors frequently show clinical symptoms and develop distant metastases more often than those with neuroendocrine tumors of other organs. More than two-third of them occur in the terminal ileum within 60 cm of ileocecal valve. Small intestinal neuroendocrine tumor is the most common small bowel malignancy. Only type I and type II are gastrin dependent. ![]() Gastric neuroendocrine tumors arise from enterochromaffin like cells. ![]() World Health Organization updated the classification of neuroendocrine tumors in 2017 and renamed mixed adenoneuroendocrine carcinoma into mixed neuroendocrine neoplasm. Most of the gastrointestinal neuroendocrine tumors are non-functional. Serum chromogranin A is the most commonly used biomarker to assess the bulk of disease and monitor treatment and is raised in both functioning and non-functioning neuroendocrine tumors. Ki-67 index and mitotic index correlate with cellular proliferation. ![]() They contain chromogranin A, synaptophysin and neuron-specific enolase which are necessary for making a diagnosis of neuroendocrine tumor. Gastrointestinal neuroendocrine tumors are rare slow-growing tumors with distinct histological, biological, and clinical characteristics that have increased in incidence and prevalence within the last few decades.
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