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The incidence of neuroendocrine tumors (NET) has increased worldwide by 3-5 times over the last decades. This is mainly based on the broad use of imaging modalities such as computed tomography (CT) and endoscopic approaches. As a consequence many duodenal and pancreatic tumors are detected in an early stage resulting in an improved prognosis of these patients. Besides the measurement of serum chromogranin A and 5-hydroxy indolic acid measured in 24 h urine collection, CT, endosonographic ultrasound (EUS) and endoscopy are important diagnostic tools. About 20% of all patients with pancreatic and duodenal NETs are diagnosed because of specific symptoms. More than 95% of diagnosed NETs are sporadic tumors. Whenever possible these patients should be treated by resection. Benign neuroendocrine duodenal tumors up to 1 cm in size can be removed endoscopically. The endoscopic resection of larger tumors should be performed surgically. The therapy of hereditary NETs of the duodenum and the pancreas should be decided after interdisciplinary discussion. However, even these patients seem to benefit from resection. In case of metastatic disease debulking surgery should be considered if more than 90% of the tumor mass can be resected. In patients with extensive liver metastases but resectable primary NET, liver transplantation is a reasonable option. There is no consensus about adjuvant or neoadjuvant treatment of duodenal or pancreatic NETs. The therapy with everolimus or sunitinib in advanced tumor stages has shown promising results. The administration of somatostatin analogues or antacids is appropriate for symptom reduction.
Klinik für Allgemein-, Viszeral- und Transplantationschirurgie, Ruprecht-Karls-Universität Heidelberg, Im Neuenheimer Feld 110, 69120, Heidelberg, Deutschland.
This article was published in the following journal.
Name: Der Chirurg; Zeitschrift fur alle Gebiete der operativen Medizen
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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.
A group of carcinomas which share a characteristic morphology, often being composed of clusters and trabecular sheets of round "blue cells", granular chromatin, and an attenuated rim of poorly demarcated cytoplasm. Neuroendocrine tumors include carcinoids, small ("oat") cell carcinomas, medullary carcinoma of the thyroid, Merkel cell tumor, cutaneous neuroendocrine carcinoma, pancreatic islet cell tumors, and pheochromocytoma. Neurosecretory granules are found within the tumor cells. (Segen, Dictionary of Modern Medicine, 1992)
The major component (about 80%) of the PANCREAS composed of acinar functional units of tubular and spherical cells. The acinar cells synthesize and secrete several digestive enzymes such as TRYPSINOGEN; LIPASE; AMYLASE; and RIBONUCLEASE. Secretion from the exocrine pancreas drains into the pancreatic ductal system and empties into the DUODENUM.
A 38-kDa integral membrane glycoprotein of the presynaptic vesicles in neuron and neuroendocrine cells. It is expressed by a variety of normal and neoplastic neuroendocrine cells and is therefore used as an immunocytochemical marker for neuroendocrine differentiation in various tumors. In ALZHEIMER DISEASE and other dementing disorders, there is an important synapse loss due in part to a decrease of synaptophysin in the presynaptic vesicles.
A GASTRIN-secreting neuroendocrine tumor of the non-beta ISLET CELLS, the GASTRIN-SECRETING CELLS. This type of tumor is primarily located in the PANCREAS or the DUODENUM. Majority of gastrinomas are malignant. They metastasize to the LIVER; LYMPH NODES; and BONE but rarely elsewhere. The presence of gastrinoma is one of three requirements to be met for identification of ZOLLINGER-ELLISON SYNDROME, which sometimes occurs in families with MULTIPLE ENDOCRINE NEOPLASIA TYPE 1; (MEN 1).
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