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Glucagonoma

Dr Jeremy Jones and Dr Frank Gaillard et al.

Glucagonomas are pancreatic tumours that secrete glucagon. Most lesions are malignant.

Epidemiology

Present with equal incidence in middle-aged men and women.

Clinical presentation

Most patients present with a necrolytic migratory rash and various other elements of the glucagonoma syndrome which includes:

diabetes mellitus
stomatitis
diarrhoea
anaemia
weight loss

Radiographic features

Tumour size is variable, but most are large and have metastasized at the time of diagnosis. Most are located in the distal pancreas and are vascular.

Tumors may be solid or contain central low-attenuation areas on CT.

Treatment and prognosis

Approximately 50% of patients survive at least 5 years after diagnosis.

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