Glucagonoma

Glucagonoma

 

Glucagonomas are neuroendocrine tumors that produce excessive amounts of glucagon and are associated with a distinctive syndrome.

Epidemiology

  • Incidence – <1/million
  • Age – median age is 50-70 years
  • Sex – equal gender distribution
  • Occurrence – sporadic most of the time

Risk Factors

  • Genetic – rarely associated with MEN 1

Pathophysiology

  • Tumor of the alpha cells of the pancreatic islets
    • Most frequently in the body and tail of the pancreas
  • Tumor is usually large when discovered
    • Most tumors are malignant

Clinical Presentation

  • Diabetes mellitus, skin rash, hypoaminoacidemia, tendency for deep vein thrombosis (DVT)
  • Skin rash
    • Migratory necrolytic erythema
    • Starts as annular erythema at intertriginous sites
    • Rash progresses to papulobullous stage that waxes and wanes
  • Frequently metastatic at presentation – liver is the most common site

Diagnosis

  • Laboratory testing
    • Glucagon level >500 pg/mL
  • Imaging
    • CT and/or MRI

Differential Diagnosis

  • Diabetes mellitus (type 1 or type 2)
  • Thrombophilic syndrome
  • Hereditary thrombophilia
  • Paraneoplastic syndromes

See Also