Malignancy in choledochal cysts

Background/Aims: Malignancy in choledochal cysts is a rare condition. This study presents our experience with this condition, with emphasis on the clinical presentation, management and outcome. Methodology: Subjects included 80 adults with choledochal cysts treated from January 1979 to December 1995. Of these patients, 8 were found to have malignancy in the cyst and formed the basis of this study. Results: Four patients had synchronous and 4 had metachronous carcinoma lesions arising in the choledochal cyst. The clinical presentation was: biliary tract infection in 5 patients, gastric outlet obstruction in 2 and right upper quadrant pain and body weight loss in 1. Operations for bile duct malignancy included total excision in 2 patients, choledochotomy with T-tube drainage in 2 patients, gastrojejunostomy in 2 patients, percutaneous transhepatic biliary drainage and gastrojejunostomy in 1 patient and metastatic lymph node biopsy only in 1. One patient died due to septic shock within 30 days of the operation (operative mortality). Postoperative survival time ranged from 4-13 months with a mean of 6.2 months. Conclusions: The frequency of malignancy in the choledochal cysts was 10% in the present series. Malignancy in the choledochal cyst should be highly suspected in patients with cholangitis symptoms, body weight loss and anemia. Prognosis in this disease entity is poor.