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Glucagonoma (alpha cell tumors)

Reviewer: Deepali Jain, M.D. (see Reviewers page)
Revised: 8 December 2012, last major update August 2012
Copyright: (c) 2001-2012, PathologyOutlines.com, Inc.


● Rare glucagon producing endocrine neoplasm arising from alpha cells of pancreas; often displays vascular invasion and may metastasize (WHO)
● Adult females, age 40+

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

Necrolytic migratory erythema

Glucagonoma in body of pancreas at surgery

Gross images

Glucagonoma of pancreatic body

Micro images

70 year old woman with glucagonoma syndrome

Gyriform festoons separated by highly vascular stroma in a clinically nonfunctioning glycogen+ adenoma

Characteristic partly monolayered gyriform pattern; malignancy cannot be determined from morphology

Incidental tumor has gyriform pattern, and was glucagon+

Most tumor cells in this gyriform, nonfunctioning alpha cell adenoma contain argyrophilic granules

MEN1 patient with dysplastic lesion mostly composed of glucagon+ cells arranged in gyriform trabeculae separated by sclerotic strands of connective tissue; note close contact, in right middle, with small glucagon negative endocrine microlobule, a remnant of the original islet

MEN1 case - glucagon cell microadenoma with trabecular-gyriform structure in surviving pancreatic lobule (bottom right); islet crowding in area of exocrine tissue atrophy due to chronic pancreatitis (upper left)

MEN1 patient with nonfunctioning glucagon-producing microadenoma

Incidental tumor at autopsy is glucagon+

Minute microadenoma mainly composed of glucagon+ cells forming gyriform trabeculae

Glucagon+ tumor

Liver metastases of malignant glucagonoma with a trabecular, partly gyriform pattern

Liver metastases are chromogranin+

Electron microscopy description

● Large, dense peripheral nucleoid

Electron microscopy images

Characteristic alpha granules with a central dense core and a peripheral, less dense mantle

Diagnostic secretory granules with a central dense core encircled by a less dense matrix

Nondiagnostic, round, homogeneous dense granules

End of Pancreas > Tumors > Glucagonoma (alpha cell tumors)

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