Pancreas
Congenital anomalies
Cystic fibrosis
Author: Deepali Jain, M.D.
Last author update: 1 August 2012
Last staff update: 30 August 2022
Copyright: 2002-2024, PathologyOutlines.com, Inc.
PubMed Search: Cystic fibrosis pancreas
Table of Contents
Definition / general | Gross images | Microscopic (histologic) description | Positive stains | Videos | Differential diagnosisCite this page: Jain D. Cystic fibrosis. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/pancreascysticfibrosis.html. Accessed December 22nd, 2024.
Definition / general
- Autosomal recessive disorder affecting most critically the lungs; also pancreas, liver, intestines; characterized by abnormal transport of chloride and sodium across an epithelium, leading to thick, viscous secretions (Wikipedia: Cystic Fibrosis [Accessed 11 December 2017])
- Incidence: 1 in 20 in U.S. are carriers
- Most common mutation is #708 (seen in 70% with disease)
- Mutations cause reduced chloride ion in secretions, thicker respiratory secretions, upper respiratory infections
- Complications: pancreatic insufficiency late in disease course in 90%, diabetes, malabsorption, pancreatitis
- Mutations also cause defective cilia and infertility; may cause meconium ileus (5 - 10%), intussusception
- Cysts form secondary to ductal obstruction due to thick, tenacious secretions, variable numbers of cysts range from 1 to 3 mm, very rarely entire pancreas replaced by multiple macroscopic cysts, termed "pancreatic cystosis"
Gross images
Images hosted on other servers:
At autopsy, pancreas
is mucoid and
slightly smaller
than normal
Microscopic (histologic) description
- Grade I: accumulation of secretion
- Grade II: exocrine atrophy
- Grade III: atrophy with lipomatosis
- Grade IV: fibrosis with total obliteration of the exocrine glands and ducts with scattered islets of Langerhans
- Pancreatic ducts diffusely dilated and filled with numerous lamellated concretions
- Associated with fibrosis
- Nondiabetic patients have fibrocystic changes with normal islets, prominent nesidioblastosis, some persisting exocrine tissue
- Young adult diabetic patients have total loss of exocrine pancreas with fat replacement, no nesidioblastosis, reduced islets (Hum Pathol 1984;15:278)
Positive stains
- PAS+ diastase resistant, CEA, alpha-1-antitrypsin (Arch Pathol Lab Med 1989;113:1142)
Videos
Histopathology pancreas: cystic fibrosis
Differential diagnosis
- Kartagener syndrome:
- Defective cilia syndrome
