Pancreas
Congenital anomalies
Familial fibrocystic pancreatic atrophy with endocrine cell hyperplasia and pancreatic carcinoma

Author: Deepali Jain, M.D. (see Authors page)

Revised: 7 December 2017, last major update August 2012

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PubMed Search: Fibrocystic pancreatic atrophy

Cite this page: Jain, D. Familial fibrocystic pancreatic atrophy with endocrine cell hyperplasia and pancreatic carcinoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/pancreasfamilial.html. Accessed October 19th, 2018.
Definition / general
  • Autosomal dominant, high penetrance, associated with diabetes mellitus, exocrine pancreatic insufficiency, characteristic imaging abnormalities
Gross description
  • Fibrosis, intraductal calcifications, microcysts
Microscopic (histologic) description
  • 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)