title: Risk of subsequent biliary malignancy in patients undergoing cyst excision for congenital choledochal cysts.
abstract: OBJECTIVE
  The aim of this study was to elucidate the risk of subsequent biliary malignancy in patients undergoing cyst excision for congenital choledochal cysts.
  
  
  METHODS
  A retrospective analysis of 94 patients who had undergone cyst excision for congenital choledochal cysts was conducted. The median age at the time of cyst excision and median follow-up time after cyst excision were 7 years and 181 months, respectively.
  
  
  RESULTS
  Biliary tract cancer developed in four patients at 13, 15, 23, and 32 years after cyst excision. The cumulative incidences of biliary tract cancer at 15, 20, and 25 years after cyst excision were 1.6%, 3.9%, and 11.3%, respectively. The sites of biliary tract cancer were the intrahepatic (n = 2), hilar (n = 1), and intrapancreatic (n = 1) bile ducts. Of the four patients with biliary tract cancer after cyst excision, three patients underwent surgical resection and one patient received chemo-radiotherapy. The overall cumulative survival rates after treatment in the four patients with biliary tract cancer were 50% at 2 years and 25% at 3 years, with a median survival time of 15 months.
  
  
  CONCLUSIONS
  The risk of subsequent biliary malignancy in patients undergoing cyst excision for congenital choledochal cysts seems to be relatively high in the long-term. The risk of biliary malignancy in the remnant bile duct increases more than 15 years after cyst excision. Despite an aggressive treatment approach for this condition, subsequent biliary malignancy following cyst excision for congenital choledochal cysts shows an unfavorable outcome.
authors.affiliation: Division of Digestive and General Surgery, Niigata University Graduate School of Medical and Dental Sciences, Niigata, Japan.
authors.forename: Taku
authors.initials: T
authors.lastname: Ohashi
authors.forename: Toshifumi
authors.initials: T
authors.lastname: Wakai
authors.forename: Masayuki
authors.initials: M
authors.lastname: Kubota
authors.forename: Yasunobu
authors.initials: Y
authors.lastname: Matsuda
authors.forename: Yuhki
authors.initials: Y
authors.lastname: Arai
authors.forename: Toshiyuki
authors.initials: T
authors.lastname: Ohyama
authors.forename: Kengo
authors.initials: K
authors.lastname: Nakaya
authors.forename: Naoki
authors.initials: N
authors.lastname: Okuyama
authors.forename: Jun
authors.initials: J
authors.lastname: Sakata
authors.forename: Yoshio
authors.initials: Y
authors.lastname: Shirai
authors.forename: Yoichi
authors.initials: Y
authors.lastname: Ajioka
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D001661:Biliary Tract Neoplasms / Q000453:epidemiology* / Q000401:mortality / Q000628:therapy; D001662:Biliary Tract Surgical Procedures / Q000009:adverse effects*; D059248:Chemoradiotherapy; D002648:Child; D002675:Child, Preschool; D015529:Choledochal Cyst / Q000453:epidemiology / Q000601:surgery*; D005260:Female; D006801:Humans; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D007564:Japan / Q000453:epidemiology; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D016577:Pancreaticoduodenectomy; D012086:Reoperation; D012189:Retrospective Studies; D018570:Risk Assessment; D012307:Risk Factors; D013997:Time Factors; D016896:Treatment Outcome; D055815:Young Adult