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Updated: Dec 26 2021


  • Snapshot
    • A 54-year-old man presents to the clinic with complaints of unintentional weight loss over the past year. He reports losing about 20 lbs. without any significant changes to his diet or exercise routine. He also noticed a rash that developed over the past week on his right hand. A physical examination reveals an erythematous plaque involving the palmar aspect of his right hand. He denies any recent contacts, outdoor activities, changes to his detergents or lotions.
  • Introduction
    • Overview
      • rare neuroendocrine tumor of pancreatic alpha cells leading to the overproduction of glucagon
  • Epidemiology
    • Incidence
      • relatively rare
      • annual incidence of 0.01 to 0.1 new cases per 100,000
    • Demographics
      • present in patients in their 5th decade
    • Risk factors
      • family history of multiple endocrine neoplasia syndrome type 1 (MEN1)
    • Pathogenesis
      • unknown etiology, the majority of cases are sporadic
      • overproduction of glucagon from pancreatic alpha cells acts on the liver
        • increases both amino acid oxidation and gluconeogenesis from amino acid substrates
        • catabolic effect of glucagon results in hyponutrition, amino acid deficiency, and hyperglucagonemia
    • Associated conditions
      • MEN1
      • Mahvash disease
  • Presentation
    • Symptoms
      • significant weight loss
      • necrolytic migratory erythema (NME)
        • erythematous papules or plaques involving the face, perineum, and extremities that eventually enlarge and coalesce
      • chronic diarrhea
      • depression
      • diaphoresis, polyuria, and polydipsia (diabetes)
      • cheilosis
    • Physical exam
      • rash characteristic of NME
  • Studies
    • Serum labs
      • increased plasma glucagon levels (> 500 pg/mL)
      • hyperglycemia
    • Skin biopsy
      • not required to make diagnosis
      • used in the diagnosis of necrolytic migratory erythema
      • pathology revelas superficial necrolysis, separation of outer layers of the epidermis, and perivascular infiltration of lymphocytes an histocytes
  • Differential
    • Pemphigus foliaceus
      • distinguishing factors
        • presence of anti-desmoglein-1 antibodies
        • normal serum glucagon
    • Pellagra
      • distinguishing factors
        • decreased niacin levels
  • Treatment
    • Medical treatment
      • supportive care including nutritional support (e.g., TPN), amino acid, and fatty acid infusions
      • somatostatin analogs (e.g., octreotide) to inhibit the release of glucagon
    • Surgical and interventional
      • pancreatic resection
        • indicated in the minority of cases where the tumor is localized to the pancreas
      • liver-directed therapy
        • includes hepatic resection, hepatic artery embolization, radiofrequency ablation and cryoablation, and/or liver transplatation
        • indicated in the majority of cases where disease has metastasized to the liver
  • Complications
    • Metastasis
      • common sites include liver, regional lymph nodes, bone, adrenal gland, kidney, and lung
    • Deep vein thrombosis and pulmonary embolism
    • Anemia
    • Reversible dilated cardiomyopathy
  • Prognosis
    • Slow growing but the majority of cases are metastatic at diagnosis
    • Poor prognosis among patients with metastatic disease
      • 10-year event free survival rate of < 51.6%
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