# Glucagonoma

Source: https://onco.cc/cancers/glucagonoma/  
OnCo record `glucagonoma` (Cancer). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

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).

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).

## Fields

- Kind: Cancer
- Last checked: 2026-09-24
- Also known as: Glucagon-secreting pancreatic neuroendocrine tumour; Glucagonoma syndrome; Alpha-cell tumour of the pancreas
- Tags: subtype-page; wave4; rare
- 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

## Sections of this record

The page is a hub with ten sections in reading order; large sections have their own page. The same plan as JSON: https://onco.cc/api/v1/cancers/glucagonoma/sections.json

- Overview (on the hub): The TL;DR, the family this cancer belongs to, the organ, who gets it and what the state of the art is. https://onco.cc/cancers/glucagonoma/#overview
- What it is (on the hub): Anatomy, the subtypes and how they differ, how it is staged, and where advanced disease spreads. https://onco.cc/cancers/glucagonoma/#what-it-is [3 subtypes]
- Finding it (on the hub): How it shows itself, how it is confirmed, what screening exists, and the biomarkers clinicians test for. https://onco.cc/cancers/glucagonoma/#finding-it [4 biomarkers]
- Treating it (on the hub): The standard of care by setting, the medicines, surgery and radiotherapy named in it, and the regimens behind them. https://onco.cc/cancers/glucagonoma/#treating-it [1 setting, 1 decision with options]
- Evidence (on the hub): Trials recruiting now, the landmark trials, the key papers and what they mean, the latest literature, and the milestones year by year. https://onco.cc/cancers/glucagonoma/#evidence
- The science (on the hub): The molecular landscape: the targets and how often each appears, the pathways, the mechanics stages and the preclinical models. https://onco.cc/cancers/glucagonoma/#science [4 targets]
- Where you are (own page): Cases by country, the UK and NHS pathway and other country lenses, and the expert centres with trials on record. https://onco.cc/cancers/glucagonoma/where-you-are/
- Living with it (on the hub): The decisions you may face, the aids that walk through them, the warnings on record, the first sixty days and the questions to ask. https://onco.cc/cancers/glucagonoma/#living-with-it [8 questions, 6 red cards]
- What is coming (own page): Everything in development, the open problems and what is being done about them, the roadmaps, and what changed on this record. https://onco.cc/cancers/glucagonoma/coming/ [2 medicines]
- Data (own page): Every connected record, the notes, the JSON, Markdown and RDF twins, and where the record came from and when it was checked. https://onco.cc/cancers/glucagonoma/data/ [12 connected records]

## Standard of care

- All cases: Resection where possible; somatostatin analogues for the syndrome; the parent page's pathways (everolimus, sunitinib, peptide receptor radionuclide therapy) for metastatic disease. ([Pancreatic neuroendocrine tumours](https://onco.cc/cancers/pancreatic-net/), [Everolimus](https://onco.cc/drugs/everolimus/), [Sunitinib](https://onco.cc/drugs/sunitinib/), [Peptide receptor radionuclide therapy (PRRT)](https://onco.cc/technologies/prrt/))

## Sources

- Wikipedia: https://en.wikipedia.org/wiki/Glucagonoma
- NCI PDQ: pancreatic neuroendocrine (islet cell) tumour treatment: https://www.cancer.gov/types/pancreatic/neuroendocrine-tumors/treatment
- Clinical Endocrinology 2011: glucagonoma and the glucagonoma syndrome, 25 years of experience: https://doi.org/10.1111/j.1365-2265.2011.03967.x
- JEADV 2016: glucagonoma syndrome, review and update on treatment: https://doi.org/10.1111/jdv.13752

## Connected records

- cancers: [Multiple endocrine neoplasia type 1 (MEN1)](https://onco.cc/cancers/men1-syndrome/), [Neuroendocrine tumours](https://onco.cc/cancers/neuroendocrine/), [Pancreatic neuroendocrine tumours](https://onco.cc/cancers/pancreatic-net/), [Somatostatinoma](https://onco.cc/cancers/somatostatinoma/), [VIPoma](https://onco.cc/cancers/vipoma/)
- technologies: [Peptide receptor radionuclide therapy (PRRT)](https://onco.cc/technologies/prrt/)
- drugs: [Everolimus](https://onco.cc/drugs/everolimus/), [Sunitinib](https://onco.cc/drugs/sunitinib/)

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JSON: https://onco.cc/api/v1/entities/glucagonoma.json