Glucagonomas are pancreatic endocrine tumors that secrete glucagon. Most lesions are malignant.

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.

Approximately 50% of patients survive for at least 5 years post-diagnosis.

Pancreatic pathology

Article information

rID: 12911
Synonyms or Alternate Spellings:
  • Glucagonomas

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