Glucagonoma

 

Clinical Background

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

Epidemiology

  • Incidence – <1/1,000,000
  • Age – median age is 50s-60s
  • Sex – M:F, equal
  • Occurrence – third most common functional neuroendocrine tumor

Risk Factors

  • Genetic – rarely associated with MEN1 or von Hippel-Lindau (VHL) syndrome
  • Usually sporadic

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

Clinical Presentation

  • Diabetes mellitus, skin rash, hypoaminoacidemia, increased risk of 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 of metastasis

Treatment

  • Symptomatic relief
  • Somatostatin analogues
  • Resection