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Pancreas

Tumors

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.

General
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● 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
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Necrolytic migratory erythema


Glucagonoma in body of pancreas at surgery

Gross images
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Glucagonoma of pancreatic body

Micro images
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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
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● Large, dense peripheral nucleoid

Electron microscopy images
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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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