{"entity":{"id":"glucagonoma","kind":"cancer","name":"Glucagonoma","aka":["Glucagon-secreting pancreatic neuroendocrine tumour","Glucagonoma syndrome","Alpha-cell tumour of the pancreas"],"tldr":"Glucagonoma is a very rare pancreatic neuroendocrine tumour that pours out the hormone glucagon, causing weight loss, diabetes and a distinctive migrating red rash. Because the rash is so characteristic, spotting it early can lead to diagnosis before the tumour has spread to the liver; surgery is the definitive treatment and somatostatin analogues control the symptoms.","summary":"Glucagonoma is a functioning pancreatic neuroendocrine tumour of alpha cells, classified by the WHO with the other functioning pancreatic neuroendocrine tumours by the hormone secreted, and accompanied in most cases by the glucagonoma syndrome of necrolytic migratory erythema, weight loss, diabetes mellitus, diarrhoea, weakness and thrombosis (Clinical Endocrinology 2011). In the six-patient series, weight loss and necrolytic migratory erythema each occurred in 83 percent and diabetes in 66 percent, plasma glucagon was raised in all, and somatostatin analogues with an aggressive surgical approach gave symptom relief and tumour control (Clinical Endocrinology 2011). The rash is usually the initial and specific finding, though it also occurs without a tumour (pseudoglucagonoma syndrome); metastases occur late, so recognising the rash before liver spread can be life-saving (JEADV 2016).\n\nHow it differs from its parent: the pancreatic neuroendocrine tumour page covers non-functioning tumours and the shared grading and treatment; glucagonoma is defined by its hormone and its syndrome, is usually large and in the tail of the pancreas by the time it is found, and is often metastatic at diagnosis after years of unrecognised symptoms.\n\nHow common: no registry figure in the sources read; the parent page notes that functioning tumours are a minority of pancreatic neuroendocrine tumours.\n\nTreatment: surgical resection is definitive; somatostatin analogues for the syndrome; the parent page's pathways (everolimus, sunitinib, peptide receptor radionuclide therapy, liver-directed therapy) for metastatic disease (JEADV 2016; NCI PDQ).","asOf":"2026-09-24","wikipedia":"https://en.wikipedia.org/wiki/Glucagonoma","links":[{"label":"NCI PDQ: pancreatic neuroendocrine (islet cell) tumour treatment","url":"https://www.cancer.gov/types/pancreatic/neuroendocrine-tumors/treatment"},{"label":"Clinical Endocrinology 2011: glucagonoma and the glucagonoma syndrome, 25 years of experience","url":"https://doi.org/10.1111/j.1365-2265.2011.03967.x"},{"label":"JEADV 2016: glucagonoma syndrome, review and update on treatment","url":"https://doi.org/10.1111/jdv.13752"}],"tags":["subtype-page","wave4","rare"],"related":["pancreatic-net","vipoma","somatostatinoma","men1-syndrome","neuroendocrine"],"cancers":[],"sections":[],"technologies":["prrt"],"targets":[],"drugs":["everolimus","sunitinib"],"companies":[],"institutions":[],"pathways":[],"terms":[],"trials":[],"people":[],"bottlenecks":[],"keyPapers":[],"journals":[],"dependsOn":[],"notes":[],"group":"endocrine","burden":"Very rare: one endocrine centre treated six patients in 25 years, median age 53.5, with a median of 39 months from first symptom to diagnosis (Clinical Endocrinology 2011). No registry figure is in the sources read.","subtypes":["Glucagonoma with the full glucagonoma syndrome (rash, weight loss, diabetes)","Glucagonoma without rash (glucagon-secreting neuroendocrine tumour)","MEN1-associated glucagonoma"],"biomarkers":["Plasma glucagon","Necrolytic migratory erythema on skin biopsy","Chromogranin A; WHO grade (Ki-67)","Somatostatin receptor imaging"],"standardOfCare":[{"setting":"All cases","approach":"Resection where possible; somatostatin analogues for the syndrome; the parent page's pathways (everolimus, sunitinib, peptide receptor radionuclide therapy) for metastatic disease.","refs":["pancreatic-net","everolimus","sunitinib","prrt"]}],"stateOfArt":[],"history":[],"pipeline":[],"openProblems":[],"parent":"pancreatic-net"},"route":"/cancers/glucagonoma/","neighbours":{"cancer":[{"id":"men1-syndrome","kind":"cancer","name":"Multiple endocrine neoplasia type 1 (MEN1)","route":"/cancers/men1-syndrome/"},{"id":"neuroendocrine","kind":"cancer","name":"Neuroendocrine tumours","route":"/cancers/neuroendocrine/"},{"id":"pancreatic-net","kind":"cancer","name":"Pancreatic neuroendocrine tumours","route":"/cancers/pancreatic-net/"},{"id":"somatostatinoma","kind":"cancer","name":"Somatostatinoma","route":"/cancers/somatostatinoma/"},{"id":"vipoma","kind":"cancer","name":"VIPoma","route":"/cancers/vipoma/"}],"technology":[{"id":"prrt","kind":"technology","name":"Peptide receptor radionuclide therapy (PRRT)","route":"/technologies/prrt/"}],"drug":[{"id":"everolimus","kind":"drug","name":"Everolimus","route":"/drugs/everolimus/"},{"id":"sunitinib","kind":"drug","name":"Sunitinib","route":"/drugs/sunitinib/"}]}}