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

Pathogenesis

  • A glucagonoma is a rare tumor of the alpha cells of the pancreas that results in the overproduction of the hormone glucagon.
  • It causes hyperglucagonemia, zinc deficiency, fatty acid deficiency, hypoaminoacidemia that may cause necrolytic migratory erythema.
  • The postulated mechanism for necrolytic migratory erythema involves the combined effect of, and liver disease, that lead to excessive inflammation in the epidermis in response to trauma and to the necrolysis.[1][2]
  • Glucagon increases gluconeogenesis from amino acid substrates. This causes weight loss due to the catabolic action of glucagon.[3]
  • Necrolytic migratory erythema probably results from hyponutrition and amino acid deficiency.[4]
  • Diarrhea may result from the secretion of gastrin occurs with hyper glucagonoma.

Genetics

Glucagonoma may be part of multiple endocrine neoplasia type1 (MEN1). It is an autosomal dominant syndrome that is usually caused by mutations in the MEN1 gene.

Gross Pathology

  • Glucagonomas are neuroendocrine tumors derived from multipotential stem cells.
  • Glucagonomas are generally large tumors at diagnosis with a mean diameter of 5 cm, From 50 to 82% have evidence of metastatic spread at presentation.
  • Nearly all glucagonomas are located in the pancreas, 50–80% occur in the pancreatic tail, 32.2% in the body and 21.9% in the head.
  • In few patients, the location was extrapancreatic, such as in kidney, duodenum, lung, accessory pancreatic tissue.
  • Metastasis usually occurs to the liver. The most common site for distal metastases is the liver
  • Other sites are lymph nodes, bone, mesentery, lung, and adrenals.[5]
  • Tumors below 2 cm in diameter are associated with a very low chance of malignancy.

Microscopic Pathology

  • Many glucagonomas are pleomorphic36,37 with cells containing granules that stain for other peptides, most frequently pancreatic polypeptide.[6]
  • Glucagon is usually detectable within the tumor cells by immunoperoxidase staining, and glucagon mRNA may be detected.
  • Electron microscopy reveals a variable number of secretory granules, indicative of their alpha cell origin.
  • Benign tumors are usually fully granulated, whereas malignant cells have fewer granules.[7]

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References

  1. Necrolytic migratory erythema. Wikipedia. https://en.wikipedia.org/wiki/Necrolytic_migratory_erythema. Accessed on October 13, 2015.
  2. 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.
  3. Braverman IM (1982). ""Cutaneous manifestations of internal malignant tumors" by Becker, Kahn and Rothman, June 1942. Commentary: Migratory necrolytic erythema". Arch Dermatol. 118 (10): 784–98. PMID 6127984.
  4. STURZBECHER M (1963). "[8 letters of Ferdinand von HEBRAS on his contributin to Virchow's Handbuch der Speziellen Pathologie and Therapie]". Z Haut Geschlechtskr. 34: 281–6. PMID 13978995.
  5. Soga J, Yakuwa Y (1998). "Glucagonomas/diabetico-dermatogenic syndrome (DDS): a statistical evaluation of 407 reported cases". J Hepatobiliary Pancreat Surg. 5 (3): 312–9. PMID 9880781.
  6. Warner TF, Block M, Hafez GR, Mack E, Lloyd RV, Bloom SR (1983). "Glucagonomas. Ultrastructure and immunocytochemistry". Cancer. 51 (6): 1091–6. PMID 6295622.
  7. Mozell E, Stenzel P, Woltering EA, Rösch J, O'Dorisio TM (1990). "Functional endocrine tumors of the pancreas: clinical presentation, diagnosis, and treatment". Curr Probl Surg. 27 (6): 301–86. PMID 1973365.
  8. 8.0 8.1 8.2 8.3 Glucagonoma. Wikimedia Commons. https://commons.wikimedia.org/wiki/File:Confluent_epidermal_necrosis_-_high_mag.jpg
  9. Castro PG, de León AM, Trancón JG, Martínez PA, Alvarez Pérez JA, Fernández Fernández JC; et al. (2011). "Glucagonoma syndrome: a case report". J Med Case Rep. 5: 402. doi:10.1186/1752-1947-5-402. PMC 3171381. PMID 21859461.
  10. Fang S, Li S, Cai T (2014). "Glucagonoma syndrome: a case report with focus on skin disorders". Onco Targets Ther. 7: 1449–53. doi:10.2147/OTT.S66285. PMC 4140234. PMID 25152626.
  11. 11.0 11.1 11.2 Erdas E, Aste N, Pilloni L, Nicolosi A, Licheri S, Cappai A; et al. (2012). "Functioning glucagonoma associated with primary hyperparathyroidism: multiple endocrine neoplasia type 1 or incidental association?". BMC Cancer. 12: 614. doi:10.1186/1471-2407-12-614. PMC 3543729. PMID 23259638.


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