Pancreas
Neuroendocrine neoplasms
Glucagonoma (alpha cell tumors)

Author: Deepali Jain, M.D. (see Authors page)

Revised: 22 December 2017, last major update August 2012

Copyright: (c) 2002-2017, PathologyOutlines.com, Inc.

PubMed Search: Glucagonoma[TI] alpha cell tumors pancreas

Cite this page: Jain, D. Glucagonoma (alpha cell tumors). PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/pancreasglucagonoma.html. Accessed August 20th, 2018.
Definition / general

2 types:
  1. Glucagonoma syndrome:
    • High incidence of malignancy
    • Adult women with large, solitary tumors, markedly elevated serum glucagon, nondescript microscopic pattern, few immunoreactive tumor cells, atypical granules on EM
    • 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)
    • Sore red tongue, angular stomatitis
    • Severe weight loss, depression
    • Deep venous thromboses
    • May develop overwhelming infection
    • Abnormal glucose tolerance test
    • Normochromic, normocytic anemia
  2. Multiple small tumors with gyriform growth pattern:
    • Nearly always benign
    • Strongly immunoreactive for glucagon
    • Typical granules on EM
Clinical images

Images hosted on PathOut server:

AFIP images:

Necrolytic migratory erythema



Images hosted on other servers:

Necrolytic migratory erythema

Glucagonoma in body of pancreas at surgery

Gross images

Images hosted on other servers:

Glucagonoma of pancreatic body

Microscopic (histologic) images

Images hosted on PathOut server:

AFIP images:

Gyriform festoons
separated by
highly vascular
stroma

Characteristic partly monolayered gyriform pattern

Tumor has gyriform pattern and was glucagon+

Endocrine adenoma stained with grimelius silver


Endocrine tumors: histogenesis

Glucagon+ tumor

Liver metastases of malignant glucagonoma

MEN1 patient with
nonfunctioning
glucagon producing
microadenoma



Images hosted on other servers:

70 year old woman with glucagonoma syndrome

Incidental tumor at autopsy is glucagon+

Liver metastases are chromogranin+

Electron microscopy description
  • Large, dense peripheral nucleoid
Electron microscopy images

Images hosted on PathOut server:

AFIP images:

Characteristic alpha granules with a central dense core

Ultrastructural appearance of glucagonoma