Multi-system disease involving pancreas

Cystic fibrosis
  • Autosomal recessive
  • Defects of serous and mucous secretion
  • 85% have exocrine pancreatic insufficiency, steatorrhea 
  • Main duct and branch ducts obstructed by inspissated secretions
  • Acinar and ductal dilatation
  • Atrophy of pancreas
  • Fatty replacement, dystrophic calcification, pancreatic cysts
Autosomal dominant polycystic kidney disease
  • 10% patients show pancreatic cysts
Von Hippel-Lindau disease
  • Autosomal dominant
  • Renal cell carcinoma, pheochromocytoma, retinal angiomatosis, hemangioblastoma in cerebellum 
  • Common is pancreatic cysts. Serous cystic neoplasms and IPMTs may also occur
Osler-Weber-Rendu disease
  • Vascular disorder
  • Involves skin, mucous membranes and viscera 
  • GI, GU, liver and or pancreas
  • Dilated pancreatic arteries supplying a racemose collection of vessels with early draining veins