作者: ANNA Manfe , Sm Basso , Franco Lumachi
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摘要: Background Neuroendocrine tumors (NET) of the gastro-entero-pancreatic (GEP) system, which comprise nonfunctioning neuroendocrine pancreatic tumors, pancreatic islets tumors, and carcinoids, are a heterogeneous group of cancers more common in the small intestine. NET have extremely varying clinical pictures, and it has been estimated a global incidence between 2.5-5 cases/100,000 per year. Each NET, depending on its anatomical site, arises from a different neuroendocrine cell, exhibiting different functionality and biological behavior. The neuroendocrine system of the GEP has at least 16 different types of endocrine cells, that produce more than 50 amines or peptides, being the widest NET system of the whole body. The NET of the small intestine are rare, and usually asymptomatic. Gastric carcinoids accounts for 4-5% of all carcinoids, originate from enterochromaffin-like cells, which are the source of several hormonal substances, including histamine, serotonine, and chromogranin A (CgA). Because of widespread and long-term use of proton pump inhibitors, and subsequent chronic hypergastrinemia, in the last decades an increased risk of gastric carcinoids have been reported. Appendiceal endocrine tumors, found incidentally in about 0.1% of appendicectomies, are often very small and benign. Colorectal carcinoids are usually discovered on colonscopy in less than 0.1% of patients. In patients with malignant carcinoids a number of prognostic parameters have been considered, such as age, clinical symptoms related to the neoplasm, TNM staging, histological grade, as well as urinary 5-hydroxyindolacetic acid (5-HIAA), and CgA …