glucagonoma

Pathology

• tumor of pancreatic islet alpha cells
• 50-80% malignant

Clinical manifestations

• rarely diabetes
• rash (mean size of tumor with skin manifestations is 16 cm)
  • secondary to branch-chain amino acid depletion in skin
  • vulvovaginitis in women
  • migrating necrotizing dermatitis
    • intertriginous erythema, scales & erosions
  • erosions, crusting & peeling involving perineum & perioral areas
  • lesions may be generalized
• stomatitis
• glossitis
• angular cheilitis
• anemia
• diarrhea
• weight loss
• malabsorption
• neuropsychiatric symptoms

Laboratory

• serum chemistries
  • serum glucose: hyperglycemia
  • glucagon: fasting levels > 1000 ng/L makes diagnosis
  • low serum zinc
• skin biopsy
• see ARUP consult

Management

• prognosis depends on aggressiveness of tumor
  • hepatic metastases in 75% at time of diagnosis
• response to chemotherapy is generally poor
  • sunitinib & everolimus are active
  • capecitabine plus temozolomide is active
• surgical excision
  • 25-30% success because peristent metastases
  • reduces tumor mass & associated symptoms

More general terms

• amine precursor uptake & decarboxylation tumor (APUDoma)
• islet cell tumor

Additional terms

• glucagon; contains: glicentin; glicentin-related polypeptide; GRPP; oxyntomodulin; OXM; OXY; glucagon-like peptide 1; GLP-1; glucagon-like peptide 1(7-37); GLP-1(7-37); glucagon-like peptide 1(7-36); GLP-1(7-36); glucagon-like peptide 2; GLP-2 (GCG)
• migratory necrolytic erythema; glucagonoma syndrome; erythema necrolytica migrans