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- W2793480082 abstract "Gastrinoma and insulinoma are the two most common functional pancreatic neuroendocrine tumors (PanNET), but there are also various kinds of rare PanNET, including glucagonomas, vasoactive intestinal peptide-releasing tumors (VIPomas) (Verner–Morrison syndrome, pancreatic cholera, watery diarrhea, hypokalemia, and achlorhydria [WDHA] syndrome), somatostatinomas, and other PanNET that secrete calcitonin, renin, luteinizing hormone, erythropoietin and insulin-like growth factor II. The diagnosis of rare PanNET requires the demonstration of an inappropriate elevation of the specific serum hormones combined with clinical and/or laboratory evidence of oversecretion of the appropriate hormone. Most rare PanNET present with metastatic disease and the survival time of patients is determined by the growth of the tumor rather than the hormone excess state. The best treatment for rare PanNET is curative surgical resection, which needs to be performed before liver metastasis develops. Indications for surgery depend on clinical symptom control, tumor size, location, extent, malignancy, and metastatic spread. Curative surgery should be indicated whenever possible, even in the presence of metastatic disease, including resectable hepatic metastases, when the patient can tolerate the surgery. In order to achieve successful surgical resection, a tumor localization study is important. Recent advances in localization techniques, such as the selective arterial secretagogue injection (SASI) test and somatostatin receptor scintigraphy (SRS), have promoted curative resection surgery. The types of surgery include pancreaticoduodenectomy, distal pancreatectomy, tumor enucleation, and enucleation in combination with pancreatic resection depending on the location of the primary tumor. Laparoscopic resection is currently not recommended because lymph node dissection and careful inspection for invasion and metastases are needed. Both somatostatin analogs and interferon have been shown to be effective in the control of symptoms in rare PanNET. Prospective studies of mammalian target of rapamycin (mTOR) inhibitors with or without octreotide and tyrosine kinase inhibitors have shown significant prolongation of survival time of the patients. New cytotoxic chemotherapy with temozolomide and capecitabine have also been reported to be effective." @default.
- W2793480082 created "2018-03-29" @default.
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- W2793480082 date "2018-02-12" @default.
- W2793480082 modified "2023-10-14" @default.
- W2793480082 title "Rare Neuroendocrine Tumors of the Pancreas" @default.
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- W2793480082 doi "https://doi.org/10.1002/9781119188421.ch135" @default.
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