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. Materials and 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. Conclusion. 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.

Tratamiento quirúrgico de los tumores de Klatskin / Surgical treatment of Klatskin tumors (Hilar Cholangiocarcinoma)

Cholangiocarcinomas are relatively rare tumors, even that in recent years it was observed a worldwide increase of its incidence. Approximately between 60-70% of cholangiocarcinomas arise from the hilus of the liver, between 20 to 30 % in the extrahepatic biliary tree, and 5 to 10 % are intrahepatic. The hilar cholangiocarcinoma was first described by Gerard Klatskin in 1965, and that is the reason for which it is termed in such eay. The prognosis is usually discouraging. The authors comment the clinical features, etiologic, factors, diagnosis and treatment. The role of staging laparoscopy in this malignancy is described: laparoscopy is a powerful diagnostic tool in patients with intrahepatic carcinoma. Staging, resectability and outcome of patients with irresecable tumors, but at present, this therapy is reserved for selected patients and in very specialized centers.

Tumor de Klatskin / Klatskin tumor

Epidemiología, factores de riesgo, clasificación, clínica, diagnóstico, y tratamiento de este tumor maligno de la confluencia biliar. En cuanto a su diagnóstico, se describen los signos directos e indirectos que deben tenerse en cuenta al realizar una ecografía, así como algunos aspectos del diagnóstico diferencial.

Colangiocarcinoma hilar / Cholangiocarcinoma

Os autores fazem revisão sobre o tumor de Klatskin, abordando, principalmente, aspectos diagnósticos e terapêutica