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Simultaneous Glucagon and Vasoactive Intestinal Peptide Producing Pancreatic Neuroendocrine Tumors in a Patient With Multiple Endocrine Neoplasia Type 1: A Case Report and Literature Review.

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FIGURE 1. Phase 2MRI of the abdomenwith contrast reveals a nonenhancing pancreatic tail cystic lesion measuring 1.8 1.6 cm (white arrow). P ancreatic neuroendocrine tumors (PNETs) are rare, accounting for… Click to show full abstract

FIGURE 1. Phase 2MRI of the abdomenwith contrast reveals a nonenhancing pancreatic tail cystic lesion measuring 1.8 1.6 cm (white arrow). P ancreatic neuroendocrine tumors (PNETs) are rare, accounting for approximately 2% of pancreatic neoplasms. The majority (50%–75%) are nonfunctional. When functional, they secrete peptide hormones— insulin, glucagon, vasoactive intestinal peptide (VIP), and gastrin. Pancreatic neuroendocrine tumors are usually sporadic but can appear as part of 1 of 4 hereditary disorders—multiple endocrine neoplasia type 1 (MEN1), Von Hippel-Lindau disease, neurofibromatosis type 1, and tuberous sclerosis. Approximately 30% to 80% of MEN1 patients develop a PNET. Among the functional PNETs, glucagonomas and VIPomas are the least common.We herein report a case of simultaneous glucagon andVIP producing PNETs in a patient with MEN1.

Keywords: peptide; glucagon vasoactive; neuroendocrine tumors; intestinal peptide; type; vasoactive intestinal

Journal Title: Pancreas
Year Published: 2018

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