Present with equal incidence in middle-aged men and women.
Most patients present with a necrolytic migratory rash and various other elements of the glucagonoma syndrome which includes:
- diabetes mellitus
- weight loss
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.