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Small bowel (small intestine)

Congenital anomalies

Heterotopic pancreas

Reviewer: Hanni Gulwani, M.D. (see Reviewers page)
Revised: 13 December 2012, last major update August 2012
Copyright: (c) 2003-2012, PathologyOutlines.com, Inc.


● Also called adenomyoma, myoepithelial hamartoma (although without pancreatic tissue)
● Incidence of 1-14%, affects all ages but peaks in 4-6th decade
● Most common near ampulla of Vater; also stomach, jejunum
● May cause blockage of duct, leading to infection, cystic dilation and fat necrosis
● Usually incidental finding at surgery submitted for frozen section, but carcinoma may arise from pancreatic heterotopia (Arch Pathol Lab Med 1999;123:707)

Case reports

● 39 year old woman with duodenal polyp (Case of the Week #226)

Gross description

● Submucosal nodule, intramural mass
● Yellow-white, lobulated, 0.2 cm to 4 cm
● May have central mucosal dimple

Micro description

● Widely separated pancreatic acini with minimally developed ducts, well formed acini may be seen (JOP 2007;8:588, Mod Pathol 2003;16:530)
● Pancreatic heterotopia may be total, only ducts, only acinar cells (exocrine heterotopia), only islet cells (endocrine heterotopia)
● Adenomyoma: predominance of pancreatic ducts with proliferation of thick smooth muscle bundles of the muscularis around the ducts (seen in periampullary region of the duodenum)

Micro images

Various images

With acinar cell carcinoma

With ductal adenocarcinoma

Differential diagnosis

● Well-differentiated adenocarcinoma
● Neuroendocrine tumor from endocrine heterotopia
● Cystic change in pancreatic heterotopia from duplication

Additional references

Arch Pathol Lab Med 2010;134:378

End of Small bowel (small intestine) > Congenital anomalies > Heterotopic pancreas

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