Ampulla of Vater - Gangliocytic paraganglioma

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Ampulla of Vater

Benign/non-neoplastic

Gangliocytic paraganglioma

Reviewer: Hanni Gulwani, M.D. (see Reviewers page)
Revised: 27 November 2012, last major update August 2012
Copyright: (c) 2003-2012, PathologyOutlines.com, Inc.

General
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● Rare tumor of periampullary region and second part of duodenum
● Usually benign, rarely has local metastases of endocrine component but even these cases are indolent
● May recur if incompletely excised
● May derive from endodermal-neuroectodermal complexes in the embryonic ventral pancreas
● Presents with GI bleeding or incidental finding

Case reports
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● 43 year old man (Arch Pathol Lab Med 2002;126:1239)
● 56 year old man (World J Surg Oncol 2010;8:42)

Clinical images
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Periampullary submucosal tumor and surface ulcer bleeding.

Gross description
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● Usually 1-3 cm, sessile or polypoid, no capsule

Micro description
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● Unencapsulated submucosal lesion
● Triphasic, with epithelioid, spindle cell (Schwann cell like), and ganglion type cells of varying proportions resembling carcinoid tumor (endocrine type cells in compact nests and trabeculae), paraganglioma and ganglioneuroma
● Usually infiltrative pattern
● Variable stromal amyloid

Micro images
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Various images

Ganglion-like (fig 3), spindle (fig 4A) & epithelial cells (fig 4B)

Positive stains
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● Endocrine cells - pancreatic polypeptide, somatostatin
● Ganglion cells - chromogranin, synaptophysin, neuron specific enolase, somatostatin
● Spindle cells - S100

Electron microscopy description
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● Dense core granules

Differential diagnosis
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● Ganglioneuroma
● Paraganglioma
● Carcinoid
● Carcinoma

Additional references
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● In Vivo 2010;24:321, Arch Pathol Lab Med 2001;125:1098 (nasopharyngeal tumor)

End of Ampulla of Vater > Benign/non-neoplastic > Gangliocytic paraganglioma

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