Loading
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.
How common: no registry figure in the sources read; the parent page notes that functioning tumours are a minority of pancreatic neuroendocrine tumours.
Treatment: 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).
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.
Most pancreatic cancers arise in the head next to the bile duct, which is why jaundice is the presenting sign; bile duct cancers are named by where along the tree they sit.
Same organ: VIPoma, Somatostatinoma, Pancreatic ductal adenocarcinoma, Biliary tract cancer (cholangiocarcinoma), Intrahepatic cholangiocarcinoma, Extrahepatic cholangiocarcinoma (perihilar and distal), Biliary tract cancer (all types), Neuroendocrine tumours, Pancreatic neuroendocrine tumours, Grade 3 well-differentiated neuroendocrine tumour, Extrapulmonary neuroendocrine carcinoma, Gallbladder cancer, Gallbladder adenocarcinoma, Papillary carcinoma of the gallbladder, Mucinous carcinoma of the gallbladder, Adenosquamous and squamous carcinoma of the gallbladder, Neuroendocrine carcinoma of the gallbladder, Incidental gallbladder cancer (found after cholecystectomy), Carcinoma in situ and dysplasia of the gallbladder, Cystic duct carcinoma, Ampullary cancer (ampulla of Vater), Resectable pancreatic ductal adenocarcinoma, Borderline resectable pancreatic ductal adenocarcinoma, Locally advanced unresectable pancreatic ductal adenocarcinoma, Metastatic pancreatic ductal adenocarcinoma, KRAS G12C-mutant pancreatic ductal adenocarcinoma, KRAS wild-type pancreatic ductal adenocarcinoma, BRCA or PALB2-mutant pancreatic ductal adenocarcinoma, Mismatch repair deficient (MSI-high) pancreatic ductal adenocarcinoma, Pancreatic acinar cell carcinoma, Intraductal papillary mucinous neoplasm and other pancreatic cystic precursors, Pancreatoblastoma
Resection where possible; somatostatin analogues for the syndrome; the parent page's pathways (everolimus, sunitinib, peptide receptor radionuclide therapy) for metastatic disease.
Country and place are remembered in this browser only. A postcode is sent to OpenStreetMap's Nominatim service to find coordinates when you press the button; nothing else leaves your device.
Query for this cancer: (TITLE:"Glucagonoma" OR ABSTRACT:"Glucagonoma" OR TITLE:"Glucagon-secreting pancreatic neuroendocrine tumour" OR ABSTRACT:"Glucagon-secreting pancreatic neuroendocrine tumour" OR TITLE:"Glucagonoma syndrome" OR ABSTRACT:"Glucagonoma syndrome" OR TITLE:"Alpha-cell tumour of the pancreas" OR ABSTRACT:"Alpha-cell tumour of the pancreas") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Glucagonoma, not a curated reading list.
The targets of this cancer's medicines and the ones linked to it directly.
Cases by country, the UK and NHS pathway and other country lenses, and the expert centres with trials on record.
One section per setting: the options named, what each is for, the trials behind them, the recorded trade-offs and the questions to ask.
Sudden severe abdominal pain, a hard or very tender abdomen, or abdominal pain with vomiting and fever. Boxed warning for gastrointestinal perforation on bevacizumab.
Fainting, near-fainting, or an irregular or racing heartbeat; several kinase inhibitors prolong the QT interval and the labels require ECG and electrolyte monitoring.
Avoid grapefruit. Live vaccines are contraindicated.
Avoid grapefruit.
Known QT prolongation. Avoid other QT-prolonging drugs where possible; check ECG and correct potassium and magnesium before and during treatment.
7.5 mg (mild), 5 mg (moderate), 2.5 mg (severe).
See all on the product pages:EverolimusSunitinib·Printable cards in the navigator
Newly diagnosed? Read the first 60 days with Glucagonoma, then print the one-page appointment sheet with room for the answers.
Print this page for your appointment (your browser's print command). These prompts are for discussion; your clinical team knows your case.
Everything in development, the open problems and what is being done about them, the roadmaps, and what changed on this record.
Every connected record, the notes, the JSON, Markdown and RDF twins, and where the record came from and when it was checked.