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Development of biliary malignancy after cyst excision for congenital choledochal cysts: What should we do?

2013; Wiley; Volume: 28; Issue: 2 Linguagem: Inglês

10.1111/jgh.12079

1440-1746

Seung Eun Lee, Jin‐Young Jang,

Congenital Anomalies and Fetal Surgery

Journal of Gastroenterology and HepatologyVolume 28, Issue 2 p. 210-212 EditorialFree Access Development of biliary malignancy after cyst excision for congenital choledochal cysts: What should we do? Seung Eun Lee, Seung Eun Lee Department of Surgery, Chung-Ang University College of Medicine, Seoul, KoreaSearch for more papers by this authorJin-Young Jang, Corresponding Author Jin-Young Jang Department of Surgery, Seoul National University College of Medicine, Seoul, Korea Correspondence Professor Jin-Young Jang, Department of Surgery, Seoul National University College of Medicine, 28 Yeongeon-dong, Jongno-gu, Seoul 110-744, South Korea. Email: jangjy4@snu.ac.krSearch for more papers by this author Seung Eun Lee, Seung Eun Lee Department of Surgery, Chung-Ang University College of Medicine, Seoul, KoreaSearch for more papers by this authorJin-Young Jang, Corresponding Author Jin-Young Jang Department of Surgery, Seoul National University College of Medicine, Seoul, Korea Correspondence Professor Jin-Young Jang, Department of Surgery, Seoul National University College of Medicine, 28 Yeongeon-dong, Jongno-gu, Seoul 110-744, South Korea. Email: jangjy4@snu.ac.krSearch for more papers by this author First published: 22 January 2013 https://doi.org/10.1111/jgh.12079Citations: 24AboutSectionsPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat The reported prevalence of cancer associated with a primary choledochal cyst ranges from 2.5%1to 26%.2 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 study3 and 0.7% in a Japanese nationwide study,4 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.5 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 cases3, 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. Table 1. Characteristics of 54 patients with subsequent biliary malignancy following choledochal cyst excision 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 study3 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. 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