Pancreas - Familial fibrocystic pancreatic atrophy with endocrine cell hyperplasia and pancreatic carcinoma

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Familial fibrocystic pancreatic atrophy with endocrine cell hyperplasia and pancreatic carcinoma

Reviewer: Deepali Jain, M.D. (see Reviewers page)
Revised: 6 December 2012, last major update August 2012
Copyright: (c) 2001-2012, PathologyOutlines.com, Inc.

General
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● Autosomal dominant, high penetrance, associated with diabetes mellitus, exocrine pancreatic insufficiency, characteristic imaging abnormalities

Gross description
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● Fibrosis, intraductal calcifications, microcysts

Micro description
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● Lobulocentric patchy to diffuse fibrocystic changes (collagenization, microcysts) with stromal fibrosis, acinar atrophy, endocrine cell hyperplasia resembling nesidioblastosis, multifocal PanIN 2-3
● Minimal background pancreatitis
● Malignancies often unusual histology: small cell, giant cell anaplastic, cystadenocarcinoma (Am J Surg Pathol 2001;25:1047)

End of Pancreas > Tumors > Familial fibrocystic pancreatic atrophy with endocrine cell hyperplasia and pancreatic carcinoma

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