Glucagonoma

Abstract

Glucagonomas are rare neuroendocrine tumours of the alpha cells of the pancreas that present with a rash and diabetes. There are under five new cases in the United Kingdom each year. The necrolytic migratory erythema presents to dermatologists. Glucagonomas can be benign or malignant in approximately equal proportions. Despite metastatic disease, the usually responds to somatostatin analogues such as octreotide and lanreotide. Tachyphylaxis occurs, so the dose is often increased over the course of the first 2 years. Patients with the gene for multiple endocrine neoplasia type 1 have an increased risk of neuroendocrine tumours of the pancreas including glucagonomas. Because these tumours often express somatostatin receptors, imaging with Gallium 68 labelled dotatate with a PET scan. Treatment choices should be discussed at a multidisciplinary meeting. Patients with uptake on Gallium 68 scanning can be treated with radiolabelled lutetium 177 labelled dotatate. The 5-year survival for patients with glucagonomas is 50–70%.