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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To the Editor:
Pancreatic neuroendocrine tumors (PNETs) are rare, accounting for approximately 2% of pancreatic neoplasms.1 The majority (50%–75%) are nonfunctional.2 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.3 Approximately 30% to 80% of MEN1 patients develop a PNET.3 Among the functional PNETs, glucagonomas and VIPomas are the least common. We herein report a case of simultaneous glucagon and VIP producing PNETs in a patient with MEN1.

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