Congenital Pancreatic Cyst
Grace E. Kim, MD
Key Facts
Terminology
Rare benign congenital epithelial-lined cyst that is not associated with ductal obstruction or other pancreatic anomalies
Does not communicate with ductal system
Clinical Issues
Exceedingly rare
Most present before 2 years of age
Often incidental finding
Macroscopic Features
Thin-walled unilocular or multilocular cyst
Occurs anywhere in pancreas
Generally 1-2 cm in size
Microscopic Pathology
Lined by flattened cuboidal to columnar epithelium
Fibroinflammatory changes can obscure epithelial lining
 The enlarged pancreatic head mass was bisected to reveal a uniloculate cyst that did not communicate with the pancreatic duct. The thin fibrous cyst wall is smooth with focal areas of hemorrhage. |
 This low-power view of a congenital cyst shows a benign unilocular cyst with a fibrous wall and a partially denuded epithelial lining. |
TERMINOLOGY
Synonyms
Dysgenetic cyst
Definitions
Rare benign congenital epithelial-lined cyst that does not communicate with ductal system
ETIOLOGY/PATHOGENESIS
Developmental Anomaly
Thought to be caused by anomalous development of pancreatic ductal system
Not associated with ductal obstruction or other pancreatic abnormalities
CLINICAL ISSUES
Epidemiology
Incidence
Exceedingly rare
Age
May be found at any age but usually in children
Most present before 2 years of age
Gender
Female predominance
Site
Any location within pancreas
More frequently in body or tail
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