Carcinoma arising in congenital choledochal cysts

Congenital choledochal cyst is almost always associated with pancreaticobiliary maljunction and is sometimes associated with biliary carcinoma. This study aimed to investigate the clinicopathological features and surgical treatment of choledochal cyst associated with carcinoma arising in the cyst wall.

Relationship of the clinicopathological findings and surgical treatment of 8 patients with histologically proven carcinoma that had developed in the choledochal cyst wall were examined to determine mode of tumor spread, multicentric tumor origins, and coincidence with other neoplastic lesions.

Papillary adenocarcinoma (n = 5) had different clinicopathological features than tubular adenocarcinoma (n = 3). Radiologically, papillary adenocarcinoma presented as an eccentrically located polypoid mass in the cyst, while with tubular adenocarcinoma, there was evidence of bile duct stenosis with irregular thickening of the bile duct wall. Papillary adenocarcinoma was associated with extensive superficial spread (n = 1), synchronous (n = 2), or metachronous (n = 2) multicentric tumors. Pancreatoduodenectomy with (n = 1) or without (n = 1) hepatic lobectomy, and repeated resection by hepatic lobectomy (n = 2) were performed for these circumstances of tumors. Extensive or repeated resections allowed 3 patients to live longer than 2 years. Tubular adenocarcinoma was associated with scirrhously infiltrative spread and a poor prognosis.

Papillary adenocarcinoma frequently occurs in the choledochal cyst wall. Since aggressive resection offers survival benefits in papillary adenocarcinoma arising in the choledochal cyst wall, the presence of superficial spread and multicentric tumors should be identified and taken into account when planning surgery. Patients require close monitoring so that recurrent carcinoma of the remnant bile duct can be identified early.