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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