Long-term survival with carcinoma arising from a congenital choledochal cyst

We report a case of 11-year survival with carcinoma arising from a congenital choledochal cyst. A 34-year-old Japanese woman was admitted due to epigastric discomfort. Ultrasonography revealed a choledochal cyst involving a polypoid tumor. Endoscopic retrograde cholangiopancreatography revealed pancreaticobiliary maljunction in which the main pancreatic duct joined the dilated common bile duct 2 cm above the papilla of Vater. A choledochal cyst containing a polypoid tumor was resected with the regional lymph nodes. Grossly, a 2×2-cm papillary tumor was noted in the posterior wall of the choledochal cyst. Microscopically, the tumor was well-differentiated tubular adenocarcinoma invading the mucosa and fibromuscular layer. Neither anticancer chemotherapy nor radiotherapy was performed. The patient recovered uneventfully after the operation and has been well for 11 years without evidence of recurrent cancer. The good results in this patient may have been due to the early stage of the cancer.