Choledochal Cyst and Associated Malignant Tumors in Adults

Abstract

To determine the clinical features and clinical outcomes of Korean adults treated surgically for choledochal cyst.Design: Retrospective nationwide multicenter study.Setting: Fifteen university hospitals (tertiary care referral centers) located in all 7 Korean provinces.

Choledochal Cysts: Presentation, Clinical Differentiation, and Management

“…29,34,35 Additionally, APBDU is less commonly seen in choledochoceles in comparison with other types of CC, and patients are more likely to have undergone a previous cholecystectomy at the time of diagnosis. 10,17,36 In fact, given the distinct differences in presentation, clinical course, diagnosis, and pathophysiology, some authors argue that choledochoceles represent a different disease entity. 17,30,33 …”

“…5,104 Carcinogenesis is thought to occur via multistep genetic events where early K-ras and p53 mutations are seen in more than 60% of CC-related carcinomas 79,106-108 followed by a late occurring DPC-4 gene inactivation. 107 Most reported cases of malignant transformation are cholangiocarcinoma; however, gallbladder carcinoma is identified in 10% to 25% of CC-related malignancies 5,10,24,27,54 (Table 1). The presence of an APBDU is thought to play a role in carcinogenesis and hepatocellular damage due to reflux of pancreatic contents into the bile duct.…”

“…50,165,166 However, the risk of biliary malignancy remains elevated even more than 15 years after CC excision, and CC-associated biliary malignancy is associated with extremely unfavorable outcomes, with a reported median survival of 6 to 21 months. 10,52,29,63,64,72,79,167 Therefore, long-term surveillance is warranted, particularly in instances with persistent intrahepatic biliary dilatation. 10,165 Typically this consists of regular biochemical evaluation and abdominal ultrasound or cross-sectional imaging.…”

“…10,52,29,63,64,72,79,167 Therefore, long-term surveillance is warranted, particularly in instances with persistent intrahepatic biliary dilatation. 10,165 Typically this consists of regular biochemical evaluation and abdominal ultrasound or cross-sectional imaging. 3 …”

Cystic Bile Duct Dilatations and Caroli’s Disease

“…The majority of BDD affected by a cancer are types I and IV according to Todani's classifi cation [ 2 ]; a cancer can be observed in all other types of BDD, but analysis of incidence is hindered by their rarity. In an analysis of a series of 63 biliary cancers on BDD, Todani et al [ 31 ] reported a tumor incidence of 68 % in type I, 5 % in type II, 1.6 % in type III, 21 % in type IV, and 6 % in type V. Recently a retrospective Korean multicenter study [ 32 ] reports a signifi cantly higher-incidence bile duct cancer in BDD type IVA (13/40, 32.5 %) and of gallbladder cancer in type I (28/35: 80 %). Presence of an ABPJ has a 16-55 % risk of malignancy, with or without congenital bile duct dilation [ 5 ].…”