Glucagonomas are rare functional neuroendocrine tumors of the pancreas that secrete excessive glucagon, which presents with weight loss, glucose intolerance/diabetes, and necrolytic migratory erythema. Less common symptoms include diarrhea, venous thromboembolic disease, and neuropsychiatric manifestations. Most cases are sporadic, but up to 20% may be associated with multiple endocrine neoplasia syndrome type 1 (MEN1). Initial treatment is supportive and involves glycemic control and improving nutritional status. When disease burden is amenable, resection of the primary tumor and debulking of metastases are indicated.
KeywordsGlucagonoma Hyperglycemia Neuroendocrine tumor Multiple endocrine neoplasia MEN1
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