4 slides generated from the cancer page, with a quiz from the open benchmark and speaker notes that cite the sources. Arrow keys move between slides; Print gives one slide per page.
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.
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).
How 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.
| Setting | Approach | Guideline |
|---|---|---|
| All cases | Resection where possible; somatostatin analogues for the syndrome; the parent page's pathways (everolimus, sunitinib, peptide receptor radionuclide therapy) for metastatic disease. | not mapped |