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.

Colangiocarcinoma en el 2011 / Cholangiocarcinoma in 2011

Cholangiocarcinoma is a rare disease in the western World, but it comprises a diagnostic and therapeutic challenge. Its outcome strongly depends on early diagnosis and complete surgical resection. As of today there are no surgical procedures that have proved increase in the survival rate for patients with these types of tumors. Recently, liver transplant appears as a promising alternative reporting a survival rate over 80 percent in 5 years. However, it has been impossible to replicate these results in the majority of the Centers specialized in the management of this disease. The present article shows epidemiological data of the disease, diagnostic methods and options of treatment according to the staging.

El colangiocarcinoma es una patología infrecuente en el mundo occidental, pero representa un desafío diagnóstico y terapéutico. Su pronóstico es muy dependiente de la precocidad del diagnóstico y la resección quirúrgica completa. Hasta ahora no existen terapias no quirúrgicas que hayan demostrado aumentar la sobrevida para este tipo de tumores. Últimamente, el trasplante de hígado aparece como una alternativa promisoria con reportes de sobrevida por sobre el 80 por ciento a 5 años, sin embargo, estos resultados no han podido ser replicados por la mayoría de los centros con alta experiencia en el manejo de esta enfermedad. En el presente artículo se detallan los antecedentes epidemiológicos de la enfermedad, los métodos diagnósticos y opciones de tratamiento según el estadío.

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.

TNM Staging of Hilar Cholangiocarcinoma / 대한소화기학회지

Hilar cholangiocarcinoma represent the majority of cholangiocarcinoma, accounting for 40-60% of whole cases. Complete resection remains the most effective and only potentially curative therapy for cholangiocarcinoma. Important factors for resection of cholangiocarcinoma such as diagnostic methods and clinical staging has been improved. Cancer staging system should be useful for guiding treatment and predicting the chance of survival. After Bismuth-Corlette classification was reported, several staging systems has been proposed and updated to accomplish this purpose. Currently 6th ed. American Joint Committee on Cancer (AJCC) staging, 2nd ed. Japanese Society of Biliary Surgery (JSBS) classification and modified Memorial Sloan-Kettering Cancer Center (MSKCC) classification are used worldwide for staging of hilar cholangiocarcinoma. These systems consider not only the tumor extent but also local biological factors that affect the resectability, but the priority among them has not yet been evaluated and randomized studies are being expected to verify this.