Glucagonoma pathophysiology

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Parminder Dhingra, M.D. [2]

Overview

Pathophysiology

  • A glucagonoma is a rare tumor of the alpha cells of the pancreas that results in the overproduction of the hormone glucagon. Alpha cell tumors are commonly associated with glucagonoma syndrome, though similar symptoms are present in cases of pseudoglucagonoma syndrome in the absence of a glucagon-secreting tumor.[1]
  • The primary physiological effect of glucagonoma is an overproduction of the peptide hormone glucagon, which enhances blood glucose levels through the activation of catabolic processes including gluconeogenesis and lipolysis. Gluconeogenesis produces glucose from protein and amino acid materials; lipolysis is the breakdown of fat.
  • Diabetes mellitus also frequently results from the insulin and glucagon imbalance that occurs in glucagonoma.[2]
  • Pathogenesis of necrolytic migratory erythema is ill defined. Postulated mechanism for necrolytic migratory erythema involves combined effect of hyperglucagonemia, zinc deficiency, fatty acid deficiency, hypoaminoacidemia, and liver disease, that leads to excessive inflammation in the epidermis in response to trauma and to the necrolysis observed in necrolytic migratory erythema.[3][4]

Microscopic Pathology

On microscopic histopathological analysis, findings of glucagonoma are:[5]

  • Epidermal necrosis
  • Subcorneal pustules, either isolated or associated with necrosis of the epidermis
  • Confluent parakeratosis
  • Epidermal hyperplasia, and marked papillary dermal angioplasia
  • Suppurative folliculitis

References

  1. Glucagonoma. Wikipedia. https://en.wikipedia.org/wiki/Glucagonoma. Accessed on October 13, 2015.
  2. Koike N, Hatori T, Imaizumi T; et al. (2003). "Malignant glucagonoma of the pancreas diagnoses through anemia and diabetes mellitus". Journal of hepato-biliary-pancreatic surgery. 10 (1): 101–5. PMID 12918465.
  3. Necrolytic migratory erythema. Wikipedia. https://en.wikipedia.org/wiki/Necrolytic_migratory_erythema. Accessed on October 13, 2015.
  4. Mullans EA, Cohen PR (1998). "Iatrogenic necrolytic migratory erythema: a case report and review of nonglucagonoma-associated necrolytic migratory erythema". J Am Acad Dermatol. 38 (5 Pt 2): 866–73. PMID 9591806.
  5. Kheir SM, Omura EF, Grizzle WE, Herrera GA, Lee I (1986). "Histologic variation in the skin lesions of the glucagonoma syndrome". Am J Surg Pathol. 10 (7): 445–53. PMID 3014912.
  6. Glucagonoma. Wikimedia Commons. https://commons.wikimedia.org/wiki/File:Confluent_epidermal_necrosis_-_high_mag.jpg
  7. Glucagonoma. Wikimedia Commons. https://commons.wikimedia.org/wiki/File:Confluent_epidermal_necrosis_-_high_mag.jpg
  8. Glucagonoma. Wikimedia Commons. https://commons.wikimedia.org/wiki/File:Confluent_epidermal_necrosis_-_high_mag.jpg


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