Glucagonoma

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

Epidemiology

They are rare with an incidence of 0.000005%. Equal incidence in middle-aged men and women. 

Clinical presentation

Most patients present with a necrolytic migratory rash and various other elements of the 4D syndrome which includes:

  • dermatitis / necrotizing migratory erythema
  • diabetes mellitus
  • deep vein thrombosis
  • depression

Stomatitis, diarrhoea, anaemia, and weight loss may also occur.

Plasma levels of glucagons are elevated (>500 pg ml).

Radiographic features

Tumour 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 at least 5 years after diagnosis.


Related articles

Pancreatic pathology

Updating… Please wait.
Loadinganimation

Alert_accept

Error Unable to process the form. Check for errors and try again.

Alert_accept Thank you for updating your details.