State of the art and future prospects in the management of neuroendocrine tumors. 2000

K Oberg
Chairman Department of Medical Sciences, University Hospital, Uppsala, Sweden.

Neuroendocrine gastroenteropancreatic tumors are rather rare neoplasms with an incidence of 1-2 cases per 100,000 people. They show rather varying tumor biology and present sometimes distinct clinical symptoms such as flushing, diarrhoea, hypoglycemia and gastric ulcers. The biochemical diagnosis is today significantly improved by the introduction of chromogranin A as a general tumor marker, which is also useful in histopathology. Today the localization procedures include somatostatin receptor scintigraphy as the primary investigation together with CT or ultrasonography. The basis for treatment of neuroendocrine GEP tumors is not only a curative intent but merely amelioration of clinical symptoms, abrogation of tumor growth, maintaining and improvement of quality of life. Surgery has always to be considered in the treatment of neuroendocrine GEP tumors. It can be performed whenever during the course of the disease but it may be more productive in earlier stages. Liver dearterialization procedures can furthermore reduce the tumor masses in liver together with laser treatment or radiofrequency therapy. The medical treatment includes cytotoxic agents, alpha interferons and somatostatin analogues. Somatostatin analogues will always be combined with the other two alternatives to reduce clinical symptoms. Chemotherapy is particularly useful for patients with more aggressive tumors with high proliferation capacity, whereas alpha interferon is beneficial in classical midgut carcinoids with low proliferation capacity. Quite recently somatostatin based radioactive tumor targeted treatment has evolved with preliminary promising data but further studies are needed to deliniate its future role in the treatment of neuroendocrine tumors in patients.

UI MeSH Term Description Entries
D010190 Pancreatic Neoplasms Tumors or cancer of the PANCREAS. Depending on the types of ISLET CELLS present in the tumors, various hormones can be secreted: GLUCAGON from PANCREATIC ALPHA CELLS; INSULIN from PANCREATIC BETA CELLS; and SOMATOSTATIN from the SOMATOSTATIN-SECRETING CELLS. Most are malignant except the insulin-producing tumors (INSULINOMA). Cancer of Pancreas,Pancreatic Cancer,Cancer of the Pancreas,Neoplasms, Pancreatic,Pancreas Cancer,Pancreas Neoplasms,Pancreatic Acinar Carcinoma,Pancreatic Carcinoma,Acinar Carcinoma, Pancreatic,Acinar Carcinomas, Pancreatic,Cancer, Pancreas,Cancer, Pancreatic,Cancers, Pancreas,Cancers, Pancreatic,Carcinoma, Pancreatic,Carcinoma, Pancreatic Acinar,Carcinomas, Pancreatic,Carcinomas, Pancreatic Acinar,Neoplasm, Pancreas,Neoplasm, Pancreatic,Neoplasms, Pancreas,Pancreas Cancers,Pancreas Neoplasm,Pancreatic Acinar Carcinomas,Pancreatic Cancers,Pancreatic Carcinomas,Pancreatic Neoplasm
D005770 Gastrointestinal Neoplasms Tumors or cancer of the GASTROINTESTINAL TRACT, from the MOUTH to the ANAL CANAL. Gastrointestinal Cancer,Cancer of Gastrointestinal Tract,Cancer of the Gastrointestinal Tract,Neoplasms, Gastrointestinal,Cancer, Gastrointestinal,Cancers, Gastrointestinal,Gastrointestinal Cancers,Gastrointestinal Neoplasm,Gastrointestinal Tract Cancer,Gastrointestinal Tract Cancers,Neoplasm, Gastrointestinal
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D018358 Neuroendocrine Tumors Tumors whose cells possess secretory granules and originate from the neuroectoderm, i.e., the cells of the ectoblast or epiblast that program the neuroendocrine system. Common properties across most neuroendocrine tumors include ectopic hormone production (often via APUD CELLS), the presence of tumor-associated antigens, and isozyme composition. Neuroendocrine Tumor,Tumor, Neuroendocrine,Tumors, Neuroendocrine

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