Disease Directory Glucagonoma
Endocrine

Glucagonoma

Also known as: Glucagon-secreting pancreatic tumor, necrolytic migratory erythema syndrome

Prevalence

Less than 1 per million per year

Onset

Adults, typically in the fifth to sixth decade

Type

Sporadic (majority); rarely associated with MEN1

About Glucagonoma

Glucagonoma is a rare functional pancreatic neuroendocrine tumour arising from alpha cells that secrete excessive glucagon, producing a distinctive clinical syndrome dominated by necrolytic migratory erythema, a characteristic skin rash, alongside diabetes mellitus, hypoaminoacidaemia, weight loss, anaemia, and venous thrombosis. The rash, which migrates and heals centrally while expanding peripherally and frequently affects the perineum, lower abdomen, and extremities, is often the presenting feature that leads to diagnosis. More than 60–70% of glucagonomas are malignant at presentation, with hepatic metastases frequently present, rendering curative resection impossible in many patients.

Common Clinical Features

Necrolytic migratory erythema (blistering, crusting, migratory rash) Diabetes mellitus or glucose intolerance Weight loss and cachexia Hypoaminoacidaemia Normochromic normocytic anaemia Deep venous thrombosis and pulmonary embolism Abdominal pain from the primary pancreatic tumour Elevated fasting serum glucagon (typically over 500 pg/mL)

Clinical Trial Eligibility Tips

What to know before applying to Glucagonoma trials.

Fasting serum glucagon level above 500 pg/mL, combined with imaging evidence of a pancreatic mass and characteristic symptoms, is the standard diagnostic threshold used for trial enrolment.

Somatostatin receptor scintigraphy or 68Ga-DOTATATE PET scan demonstrating receptor positivity is required for trials of somatostatin analogues or peptide receptor radionuclide therapy (PRRT).

Thromboprophylaxis and nutritional status (albumin, amino acid levels) are assessed at baseline screening; patients with active deep vein thrombosis or severe malnutrition may need stabilisation before enrolment.

Patient Resources

Patient Organization

Neuroendocrine Tumor Research Foundation

Visit website ↗

Natural History Registry

NANETS Patient Registry

Join registry ↗

Orphanet

European reference resource for rare diseases (ORPHA:2015)

View on Orphanet ↗

NORD

National Organization for Rare Disorders

Search NORD ↗

Find recruiting Glucagonoma trials

Search 500,000+ studies from ClinicalTrials.gov, filtered for Glucagonoma. Updated daily.

Related Rare Diseases