Glucagonoma
Glucagon-secreting pancreatic neuroendocrine tumour
Overview
A rare glucagon-secreting pancreatic neuroendocrine tumour, classically presenting with the 4 D's: Dermatitis (NECROLYTIC MIGRATORY ERYTHEMA), Diabetes (mild), DVT/thromboembolism, Depression — plus weight loss and a sore red tongue/angular stomatitis. Diagnosed by a very high glucagon; treated with somatostatin analogues and resection.
Recognise
- NECROLYTIC MIGRATORY ERYTHEMA: migrating, crusted, erosive erythematous rash (groin/perineum/limbs) — the hallmark
- '4 D's': Dermatitis, Diabetes (mild), DVT/thromboembolism, Depression; weight loss, glossitis/angular stomatitis, anaemia
- Markedly raised plasma glucagon
Red flags
- Venous thromboembolism risk
- Diagnostic delay (rash misdiagnosed) — recognise necrolytic migratory erythema
Differentials & how to tell them apart
Investigations
Plasma GLUCAGON (very high); chromogranin A; localise (CT/MRI, somatostatin scan); glucose, zinc/amino acids (deficiency contributes to the rash).
Management
Somatostatin analogue + resection (anticoagulate; nutritional support)
- 1Recognise necrolytic migratory erythema + the 4 D's; confirm with a very high glucagon and localise. Somatostatin analogue for symptoms; resect localised disease.Gate: The migrating erosive rash (necrolytic migratory erythema) is the recognition key — and the thrombotic risk warrants thromboprophylaxis
- 2Resection; somatostatin analogue/chemotherapy for metastatic disease; manage diabetes/thrombosis/nutrition.
Key points
Necrolytic migratory erythema + mild diabetes + DVT + depression + weight loss = glucagonoma → high glucagon. The rash is the giveaway.
Monitor & prognosis
Glucagon; rash/glucose; thrombosis.
Often metastatic at diagnosis but indolent.
Source: ENETS/UKINETS