Pancreas
Neuroendocrine neoplasms
Glucagonoma (alpha cell tumors)
Author: Raul S. Gonzalez, M.D.
Editor-in-Chief: Debra L. Zynger, M.D.
Last author update: 17 February 2021
Last staff update: 21 June 2024
Copyright: 2002-2024, PathologyOutlines.com, Inc.
PubMed Search: Glucagonoma alpha cell tumor AND pancreas
Table of Contents
Definition / general | Essential features | Terminology | ICD coding | Epidemiology | Sites | Pathophysiology | Clinical features | Diagnosis | Laboratory | Radiology description | Radiology images | Case reports | Treatment | Clinical images | Gross description | Microscopic (histologic) description | Microscopic (histologic) images | Positive stains | Electron microscopy description | Sample pathology report | Differential diagnosis | Board review style question #1 | Board review style answer #1 | Board review style question #2 | Board review style answer #2Cite this page: Gonzalez RS. Glucagonoma (alpha cell tumors). PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/pancreasglucagonoma.html. Accessed November 29th, 2024.
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
- Incidence rate of approximately 2.5/100,000,000 (Oncol Lett 2018;15:2749)
- Mean age 54 years
- More common in women
Sites
- More common in tail of pancreas
Pathophysiology
- Generally sporadic
- Patients with multiple endocrine neoplasia type 1 may have multiple small glucagonomas (with better prognosis)
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
Images hosted on other servers:
CT
MRI
Liver metastases
Case reports
- 50 year old woman with pruritic rash (Oncol Lett 2015;10:1113)
- 51 year old woman with heart failure (Endocrinol Diabetes Metab Case Rep 2014;2014:140061)
- 62 year old woman with ovarian mass and history of glucagonoma (JOP 2013;14:510)
- 64 year old woman with erythematous rash (Case Rep Surg 2016;2016:1484089)
- 68 year old man with anemia and diabetes (J Hepatobiliary Pancreat Surg 2003;10:101)
Treatment
- Surgery is only curative option
- Somatostatin analogues may relieve symptoms (Clin Endocrinol (Oxf) 2011;74:593)
Clinical images
AFIP images
Necrolytic migratory erythema
Images hosted on other servers:
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
Images hosted on other servers:
H&E and immunostains
H&E and glucagon
Liver metastasis
Chromogranin
Positive stains
- Glucagon (though this does not establish the diagnosis)
- Synaptophysin and chromogranin
Electron microscopy description
- Single type secretory granules similar to normal alpha cell granules (Ultrastruct Pathol 1989;13:15)
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
- Nonglucagon secreting pancreatic neuroendocrine tumor:
- Must be determined on clinical grounds (Proc (Bayl Univ Med Cent) 2015;28:46)
Board review style question #1
- Which of the following is a potential clinical symptom of pancreatic glucagonoma?
- Angular stomatitis
- Hypochlorhydria
- Hypoglycemia
- 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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Board review style question #2
- Which of the following is necessary to confirm a diagnosis of glucagonoma in a patient with a pancreatic mass?
- Classic radiologic findings
- Clinical glucagonoma type symptoms
- Immunohistochemical positivity for glucagon
- 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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