Choledochal cyst and neoplasm: a comprehensive review of 106 cases and presentation of two original cases.

Choledochal cysts are prone to complications: cholangitis, biliary cirrhosis, portal hypertension, lithiasis, rupture, pancreatitis, and carcinoma. The coincidence of choledochal cysts and neoplasia ranges from 2.5 to 26 per cent. One hundred six cases of choledochal cysts with neoplasms have been collected from the literature. We have tabulated the results of 68 cases found to have a neoplasm at the initial laparotomy and of 38 patients with pristine choledochal cysts treated electively who subsequently developed a neoplasm. Two original cases are presented. The primary site of neoplasia was not confined only to choledochal cysts. There appears to be a propensity for malignancy to develop anywhere in the biliary tract or gallbladder or pancreas in conjunction with the choledochal cyst. Accompanying choledochal cysts is a high incidence of an anomalous relation at the pancreaticobiliary junction with subsequent malignancy formation. A pathogenetic basis is postulated.