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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.


● 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

● 46 year old woman with WDHA (watery diarrhea/hypokalemia/achlorhydria) syndrome (Case Rep Gastroenterol 2008;2:238)

Gross images

Various images

Micro images

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

Abundant basal accumulation of secretory granules

Differential diagnosiss

● Pancreatic polypeptide cell hyperplasia: J Clin Pathol 2006;59:1087

End of Pancreas > Tumors > Pancreatic polypeptide-secreting tumors

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