title: Intrahepatic, perihilar and distal cholangiocarcinoma: Management and outcomes.
abstract: Introduction and aims. Cholangiocarcinomas are a heterogeneous group of tumors that can be classified into three clinically distinct types of cancers, intrahepatic, perihilar and distal cholangiocarcinoma. The inconsistent use of nomenclature for these cancers has obscured a true knowledge of the epidemiology, natural history and response to therapy of these cancers. Our aims were to define demographic characteristics, management and outcomes of these three distinct cancer types.
  
  
  METHODS
  A retrospective study of patients enrolled in an institutional cancer registry from 1992 to 2010. Median survival was compared between different treatment modalities over three time periods for the three types of cholangiocarcinoma at different stages of the disease using Kaplan Meyer analysis.
  
  
  RESULTS
  242 patients were identified. All cases were reviewed and classified into intrahepatic (90 patients), distal (48 patients) or perihilar (104 patients) cholangiocarcinomas. These cancers differed in median age of onset, gender distribution, median survival and stage. 13.8% of patients presented with stage I, 5.8% with stage II, 9.6% with stage III, 28% with stage IV, with 41.8% having unknown stage. The overall median survival was 15.8 months, and was 23, 25, 14, and 4.5 months for stages I, II, III, and IV respectively. Surgery improved survival in both early and advanced stages. Multimodality therapies further improved outcomes, particularly for perihilar cholangiocarcinoma.
  
  
  CONCLUSIONS
  Perihilar, distal and intrahepatic cholangiocarcinoma vary in their presentation, natural history and therapeutic approach to management. A consistently applied classification is essential for meaningful interpretation of studies of these cancers.
authors.affiliation: Department of Transplantation, Mayo Clinic Florida, USA.
authors.forename: David
authors.initials: D
authors.lastname: Waseem
authors.affiliation: Department of Transplantation, Mayo Clinic Florida, USA.
authors.forename: Patel
authors.initials: P
authors.lastname: Tushar
mesh_terms: D000368:Aged; D000970:Antineoplastic Agents / Q000009:adverse effects / Q000627:therapeutic use*; D001650:Bile Duct Neoplasms / Q000145:classification / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D001662:Biliary Tract Surgical Procedures / Q000009:adverse effects / Q000401:mortality; D017024:Chemotherapy, Adjuvant; D018281:Cholangiocarcinoma / Q000145:classification / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D005260:Female; D005431:Florida; D006801:Humans; D053208:Kaplan-Meier Estimate; D018285:Klatskin Tumor / Q000145:classification / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D008297:Male; D008875:Middle Aged; D009367:Neoplasm Staging; D018714:Radiotherapy, Adjuvant; D012042:Registries; D012189:Retrospective Studies; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D000970:Antineoplastic Agents