Pancreas

Neuroendocrine neoplasms

Glucagonoma (alpha cell tumors)


Editor-in-Chief: Debra L. Zynger, M.D.
Raul S. Gonzalez, M.D.

Topic Completed: 17 February 2021

Minor changes: 24 February 2021

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PubMed Search: Glucagonoma alpha cell tumor AND pancreas AND free full text[sb]

Raul S. Gonzalez, M.D.
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Cite this page: Gonzalez RS. Glucagonoma (alpha cell tumors). PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/pancreasglucagonoma.html. Accessed October 16th, 2021.
Definition / general
  • Rare, poorly characterized pancreatic neuroendocrine tumor that produces glucagon
Essential features
  • WHO recognized diagnosis
  • Produces glucagon, akin to alpha cells of the pancreas
  • Clinical glucagonoma symptoms include necrolytic migratory erythema, diabetes, weight loss and anemia
Terminology
  • Necrolytic migratory erythema: skin rash of legs, perineum, groin; rash becomes blisters with central clearing, heals with hyperpigmentation but without scarring in 7 - 14 days (Am J Surg Pathol 1986;10:445)
ICD coding
  • ICD-10: D37.8 - Neoplasm of uncertain behavior of pancreas
Epidemiology
Sites
  • More common in tail of pancreas
Pathophysiology
Clinical features
  • Causes glucagonoma syndrome (necrolytic migratory erythema, diabetes mellitus, weight loss, anemia, angular stomatitis)
  • Necrolytic migratory erythema may become infected
  • Roughly half of cases metastasize, often to liver
Diagnosis
  • Determined clinically, not by immunohistochemical positivity for glucagon
Laboratory
  • Elevated blood glucagon levels
Radiology description
  • MRI: low signal intensity on T1, slightly high signal intensity on T2
Radiology images

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CT

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MRI

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Liver metastases

Case reports
Treatment
Clinical images

AFIP images

Necrolytic migratory erythema



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Necrolytic migratory erythema

Gross description
  • Average gross tumor size is 5.0 cm
Microscopic (histologic) description
  • Nests of monotonous low grade neuroendocrine cells with salt and pepper nuclei and ample amphophilic cytoplasm
Microscopic (histologic) images

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H&E and immunostains

H&E and glucagon

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Liver metastasis

Chromogranin

Positive stains
Electron microscopy description
Sample pathology report
  • Pancreas and duodenum, Whipple procedure:
    • Pancreatic neuroendocrine tumor, WHO grade 1 (see synoptic report and comment)
    • Comment: The patient’s clinical history of necrolytic migratory erythema is noted. This may be due to glucagon secretion by this tumor, which would make it a glucagonoma. Immunohistochemical stains for synaptophysin and chromogranin are positive and the Ki67 index is approximately 1.2%.
Differential diagnosis
Board review style question #1
    Which of the following is a potential clinical symptom of pancreatic glucagonoma?

  1. Angular stomatitis
  2. Hypochlorhydria
  3. Hypoglycemia
  4. Water diarrhea
Board review style answer #1
A. Angular stomatitis (though the most common finding is necrolytic migratory erythema). Hypochlorhydria is sometimes seen in somatostatinoma. Hypoglycemia is typical of insulinoma. Watery diarrhea is typical of VIPoma.

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Reference: Glucagonoma (alpha cell tumors)
Board review style question #2
    Which of the following is necessary to confirm a diagnosis of glucagonoma in a patient with a pancreatic mass?

  1. Classic radiologic findings
  2. Clinical glucagonoma type symptoms
  3. Immunohistochemical positivity for glucagon
  4. Specific histologic findings
Board review style answer #2
B. Clinical glucagonoma type symptoms. The diagnosis of glucagonoma is made on clinical grounds in the setting of a pancreatic mass lesion.

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Reference: Glucagonoma (alpha cell tumors)
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