Isolated polycystic disease of pancreas

Excerpt

Dysontogenetic cyst is a well-established disease association with polycystic kidney disease, cystic fibrosis, von Hippel-Lindau disease, and Ivemark syndrome. True pancreatic cyst is a developmental abnormality of primitive pancreatic duct sequestration [1]. Isolated diffuse polycystic pancreas in adults is an unusual occurrence. Its diagnosis merits thorough search for associated pathology. Despite diffuse cystic replacement, no functional derangement or symptom related to pancreatic dysfunction is usually observed. Steatorrhea and malnutrition may manifest in infancy, are evidently the result of deficiency of pancreatic exocrine secretions. Cysts may become symptomatic secondary to mass effect from enlarging cyst or complication related to cyst rupture, infection or biliary obstruction, and pancreatitis. Major issue is to differentiate it from rare diffuse cystic neoplasm [2]. On imaging (Figs. 1 and 2), the pancreas is studded with multiple simple cysts, with no evidence of calcification, hemorrhage, or enhancing nodule. Reliable diagnosis is only made on histological finding of epithelial lined cyst wall, finding high enzyme activity within the contained fluid and negative tumor markers. Only treatment option for dominant symptomatic cyst is segmental pancreatectomy with cystectomy. Wait-and-see strategy should be adopted for asymptomatic diffuse pancreatic involvement.

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