They are very rare with an incidence of ~0.000005% or less than 1 case per 20 million. Equal incidence in middle-aged men and women.
Most patients present with a necrolytic migratory rash and various other elements of the 4D syndrome which includes:
Stomatitis, diarrhea, anemia, and weight loss may also occur.
Plasma level of glucagon is elevated (>500 pg/mL).
Tumor size is variable, but most are large (>5 cm) 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 for at least 5 years post-diagnosis.
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