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 June 18th, 2019.
Definition / general
- Autosomal dominant, high penetrance, associated with diabetes mellitus, exocrine pancreatic insufficiency, characteristic imaging abnormalities
- 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)