Development of biliary malignancy after cyst excision for congenital choledochal cysts: What should we do?
Article first published online: 22 JAN 2013
© 2013 Journal of Gastroenterology and Hepatology Foundation and Wiley Publishing Asia Pty Ltd
Journal of Gastroenterology and Hepatology
Volume 28, Issue 2, pages 210–212, February 2013
How to Cite
See article in J. Gastroenterol. Hepatol. 2013; 28: 243–247, 28:2, 210–212.
- Issue published online: 22 JAN 2013
- Article first published online: 22 JAN 2013
- Manuscript Accepted: 28 NOV 2012
The reported prevalence of cancer associated with a primary choledochal cyst ranges from 2.5%to 26%. Total cyst excision is the standard procedure to prevent malignant changes. An essential part of this surgery is the termination of the reflux of pancreatic juice into the bile duct through the anomalous pancreaticobiliary duct union, so as to avoid activation of pancreatic enzymes involved in the pathogenesis of bile duct carcinoma. Theoretically, biliary malignancy should not develop after excision of the choledochal cyst because the presumed cause was abrogated by separation-operation. However, although the incidence of biliary malignancy after excision of choledochal cyst was 0.6% in Korean multicenter study and 0.7% in a Japanese nationwide study, far lower than that associated with unresected choledochal cysts, it is still higher (about 200 times) than the incidence of biliary cancer in the general population in Japan. The main issues about the biliary malignancy after cyst excision are the perspective it might give on risk factors for oncogenesis, the relevance of cyst excision as a standard treatment and risk factors.
In this issue of the Journal of Gastroenterology and Hepatology, Ohashi et al. provided cumulative incidence data on subsequent biliary malignancy increase more than 15 years after choledochal cyst excision, describe unfavorable outcomes, and provide a comprehensive review of biliary malignancy after cyst excision. However, several questions mentioned remained unanswered.
The authors searched only the English literature. Because choledochal cyst is a relatively rare disease in the West and more than half of the reported cases have occurred in Japan, we searched both English and Japanese language literature. Between 1970 and 2011, 58 cases were identified, and among them, data of site of malignancy can be available in 54 cases[3, 6-47] (Table 1). According to Todani's classification, 24 of 41 patients (59%) were classified into type IVa, and 17 (42%) were into type I. The most common site of involvement in 54 cases was the hepatic duct, at or near the choledocho-enteric anastomosis (43%) followed by the intrahepatic duct (41%) and distal choledochus (17%). The time between cyst excision and cancer detection ranged from 1 to 32 years (mean 10.4 years). Interestingly, a fair number of patients gave a medical history of cholangitis or hepatolithiasis after choledochal cyst excision. It seems that postoperative or pre-existed stenosis of bile duct, bile stasis caused by stenosis, and repeated chronic inflammation of the epithelium might induce carcinogenesis. Therefore, wide anastomosis with free drainage of bile as well as complete excision of dilated bile duct appears essential to prevent development of carcinoma.
|Intrahepatic bile duct (n = 22)||Hilar or near anastomosis (n = 23)||Intrapancreatic remnant bile duct (n = 9)||Total (n = 54)|
|M : F||1:1.25||1:2.2||1:8||1:2.1|
|Type of choledochal cyst (I/IVA)||7:10||6:11||4:3||17:24|
|Age at cyst excision (mean, year, range)||30.2 (1–51)||28.7 (1–50)||23.4 (8–70)||29.0 (1–70)|
|Age at detection of biliary malignancy (mean, year, range)||39.1 (19–72)||40.0 (18–66)||32.7 (23–72)||39.8 (18–72)|
|Interval between cyst excision and cancer detection (mean, year, range)||10.2 (1–32)||10.0 (2–21)||9.0 (4–17)||10.4 (1–32)|
|Resection of malignant lesion||3/15 (20.0%)||8/11 (72.7%)||2/5 (40.0%)||13/31 (41.9%)|
|Cholangitis/hepatolithiasis after cyst excision||9 (40.9%)||9 (39.1%)||2 (22.2%)||20 (37.4%)|
The prognosis of the above 54 cases was grimmer than that of cholangiocarcinoma in general, with a survival from 1 to 30 months. The reason for this poor prognosis is believed to be a low rate of resectability after diagnosis at an advanced stage. However, a Korean multicenter study showed more than 70% of patients underwent curative resection because of widespread careful long-term follow-up and relatively early detection. About 60% of patients were classified as stage I or II, and the 5-year survival rates were comparable with that of cholangiocarcinoma in general.
Although carcinogenesis associated with choledochal cyst is still unresolved, we have learned several things about this issue. Initially, complete excision of the dilated bile duct at the level of confluence with the pancreatic duct and wide anastomosis with free drainage of bile should be performed. Thereafter, lifelong regular follow-up through tumor marker such as serum level of CA19-9 and imaging modalities such as computed tomography or ultrasonography for early detection of subsequent biliary malignancy after cyst excision should be done. Should recurrent cholangitis or hepatolithiasis occur, early treatment should be done as well as efforts to find the stenotic site and to correct such stenosis as early as possible.
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