Choledochal cyst: Anomalous arrangement of the pancreaticobiliary ductal system and biliary malignancy

Abstract

Congenital biliary dilatation (CBD) or choledochal cyst has previously been considered a rarity; however, the number of cases reported in the literature has been increasing. It is generally accepted that there is a predominance in Japanese, as more than one‐third of the cases are from Japanese literature.

Ki-ras point mutations and proliferation activity in biliary tract carcinomas

“…As a further possible factor influencing mutations in BTC, especially of significance for GBC, anomalous arrangement of the pancreaticobiliary ductal system (APB), which permits reflux of pancreatic juice into the biliary system has been recognised (Komi et al, 1989). The interaction of pancreatic juice with bile has been reported to play a key role in cholangiocarcinogenesis (Qian et al, 1993).…”

A case of metachronous biliary carcinoma

“…It is tempting to speculate that these patients have a predisposition to multiple biliary carcinomas. Some of these multiple tumours are associated with pancreaticobiliary maljunction [2]. This patient had a DCBD.…”

Hepatopancreatoduodenectomy for Diffuse Bile Duct Carcinoma Arising in Choledochal Cyst

“…Thus, it seems that detection of a complicating malignancy in a choledochal cyst is difficult at a relatively early stage, and that prognosis after surgery is dismal, even if bile duct carcinoma is successfully diagnosed preoperatively. Accordingly, excision of the dilated biliary tract with hepaticoenterostomy should be carried out as early as possible [ 1 ]. Otherwise, early detection of the car cinoma is mandatory, although this is more difficult than for a bile duct carcionoma without choledochal cyst, because any symptoms resulting from the carcinoma will not become manifest without progression of the cancer due to the wide cavity of the choledochal cyst.…”

“…The incidence of the choledochal cyst is generally accepted to be relatively high among the Japanese, and those with this condition have a high risk of biliary malig nancy compared with the general adult population [1]. A low resectability rate of carcinoma arising in the choledo chal cyst and a poor prognosis have been reported [2,3].…”