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Pancreas
Endocrine tumors
Pancreatic polypeptide-secreting 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 endocrine neoplasm with high proportion of pancreatic polypeptide (PP) cells, which typically are more frequent in posterior head of pancreas (from ventral bud)
● Not a WHO diagnosis
● Asymptomatic despite high levels of the hormone in plasma
● Note: PP cells are common in pancreatic endocrine tumors of other types
Case reports
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● 46 year old woman with WDHA (watery diarrhea/hypokalemia/achlorhydria) syndrome (Case Rep Gastroenterol 2008;2:238)
Gross images
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Micro images
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A: PP-oma showing abundance of PP immunoreactive cells (in black); B-predominance of PP immunoreactive cells
Nonfunctioning PP-cell tumor showing ribbons and festoons of tumor cells present throughout the neoplasm, separated by loose fibrovascular stroma
PP+ tumor has partly trabecular structure (left) with palisading of cells, and partly a solid pattern; tumor was large (5.5 cm in diameter) and associated with clinical symptoms due to local compression; tumor cells are pancreatic polypeptide+ (right)
Electron microscopy images
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Abundant basal accumulation of secretory granules
Differential diagnosiss
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● Pancreatic polypeptide cell hyperplasia: J Clin Pathol 2006;59:1087
End of Pancreas > Tumors > Pancreatic polypeptide-secreting tumors
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