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, eight were found to have malignancy in the cyst and formed the basis of this study.
Results: Four patients had synchronous and four had metachronous carcinoma lesions arising in the choledochal cyst. The clinical presentations were: biliary tract infection in 5 patients, gastric outlet obstruction in two and right upper quadrant pain and body weight loss in one. Operations for bile duct malignancy included total excision in two patients, choledochotomy with T-tube drainage in two patients, gastrojejunostomy in two patients, percutaneous transhepatic biliary drainage and gastrojejunostomy in one patient and metastatic lymph node biopsy only in one. One patient died due to septic shock within 30 days of operation (operative mortality). Postoperative survival time ranged from 4 to 9 months with a mean of 6.2 months. One of these patients was still alive at the time of writing (7 months).
Conclusions: The frequency of malignancy in the choledochal cysts was 10% in our 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.