Congenital Pancreatic Cyst

 Presumably due to failure of embryonic pancreatic ducts to regress, when they are replaced by permanent ones, that leads to their obstruction and cyst formation in utero

image May be associated with other congenital anomalies, such as von Hippel-Lindau disease, polycystic kidney disease, etc.

Clinical Issues

• Exceedingly rare

• May be found at any age but usually in children younger than 2 years

• Often incidental finding, but may show signs and symptoms of GI or biliary obstruction or pancreatitis due to pressure on adjacent organs

• Any location within pancreas but more common in body or tail


• Usually single, unilocular, thin-walled cystic lesion
image Typically 1-2 cm in size but can be larger

• Can be multiple, multilocular
image May diffusely involve entire pancreas

image More common in patients with von Hippel-Lindau or polycystic kidney disease

• Does not communicate with pancreatic ductal system


• Cysts are lined by single layer of nonmucin-producing epithelium that can be flattened, cuboidal to columnar

• Fibrous or fibroinflammatory cyst wall

Top Differential Diagnoses

• Enteric duplication cyst

• Retention cyst

• Pseudocyst

• Serous cystadenoma

Unilocular Cyst
The enlarged pancreatic head mass was bisected to reveal a unilocular cyst image that did not communicate with the pancreatic duct. The thin fibrous cyst wall is smooth with focal areas of hemorrhage.

Flattened Epithelial Lining
This low-power view of a congenital pancreatic cyst shows a unilocular cyst lined by a single layer of attenuated epithelium image. The cyst wall is fibrotic.

Epithelial Lining
The cystic space in a congenital pancreatic cyst is typically lined by a single layer of flattened, cuboidal or columnar nonmucinous epithelium image, similar to that seen in benign pancreatic ducts.

Cyst Wall
The wall of a congenital pancreatic cyst typically shows fibrosis and may contain varying degrees of inflammation consisting mainly of lymphocytes image .



• Dysgenetic cyst


• Rare, benign, congenital, epithelial-lined intrapancreatic cyst that does not communicate with ductal system

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Apr 20, 2017 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Congenital Pancreatic Cyst

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