title: Intrahepatic cholangiocarcinoma in a low endemic area: rising incidence and improved survival.
abstract: BACKGROUND
  To explore trends in the incidence and survival of patients with intrahepatic cholangiocarcinoma (ICC) an unselected population in Western Europe was studied.
  
  
  METHODS
  Between 1989 and 2009, all patients newly diagnosed with ICC were selected from the Netherlands Cancer Registry (n= 809). Trends in incidence, treatment and relative survival were calculated according to gender and age. Follow-up for vital status was complete until 1st January 2010.
  
  
  RESULTS
  The incidence rates of ICC increased significantly between 1999 and 2009, especially in the age group 45-59 years [estimated annual percentage change +3.0%, 95% confidence interval (CI) 0.2-5.8]. In the other age groups ICC incidence remained stable. Patients diagnosed with tumour lymph node metastasis (TNM) stage I mainly underwent surgery (68%), and the majority of the patients with stage II, III and IV received best supportive care (73%). One-year relative survival for patients with ICC increased significantly from 24% in 1989-1994 to 28% in 2005-2009 (P= 0.03), and corresponding 3-year relative survival improved from 4% to 8% (P= 0.02). Three-month and 1-year relative survival for patients with ICC receiving surgery was 91% and 71%, respectively.
  
  
  CONCLUSIONS
  Between 1999 and 2009, the incidence of ICC rose, especially in the age group 45-59 years, suggesting aetiological influences. Survival rates have improved during the study period.
authors.affiliation: Department of Hepatobiliary and Transplantation Surgery, Erasmus MC, University Medical Centre, Rotterdam, The Netherlands.
authors.forename: Caroline D M
authors.initials: CD
authors.lastname: Witjes
authors.forename: Henrike E
authors.initials: HE
authors.lastname: Karim-Kos
authors.forename: Otto
authors.initials: O
authors.lastname: Visser
authors.forename: Esther
authors.initials: E
authors.lastname: de Vries
authors.forename: Jan N M
authors.initials: JN
authors.lastname: IJzermans
authors.forename: Robert A
authors.initials: RA
authors.lastname: de Man
authors.forename: Jan Willem W
authors.initials: JW
authors.lastname: Coebergh
authors.forename: Cornelis
authors.initials: C
authors.lastname: Verhoef
mesh_terms: D000328:Adult; D017677:Age Distribution; D000367:Age Factors; D000368:Aged; D001650:Bile Duct Neoplasms; D001653:Bile Ducts, Intrahepatic; D018281:Cholangiocarcinoma / Q000453:epidemiology* / Q000401:mortality / Q000473:pathology / Q000628:therapy; D019353:Endemic Diseases; D005260:Female; D006801:Humans; D015994:Incidence; D008113:Liver Neoplasms / Q000453:epidemiology* / Q000401:mortality / Q000473:pathology / Q000628:therapy; D008207:Lymphatic Metastasis; D008297:Male; D008875:Middle Aged; D009367:Neoplasm Staging; D009426:Netherlands / Q000453:epidemiology; D011379:Prognosis; D012042:Registries; D018570:Risk Assessment; D012307:Risk Factors; D017678:Sex Distribution; D012737:Sex Factors; D016019:Survival Analysis; D013997:Time Factors