An Immunohistochemical Analysis of Osteopontin and S100 Calcium-binding Protein P is Useful for Subclassifying Large- and Small-duct Type Intrahepatic Cholangiocarcinomas.

Intrahepatic cholangiocarcinoma (iCCA) has been newly subclassified into two different subtypes: large-duct (LD) type and small-duct (SD) type. However, many cases are difficult to subclassify, and there is no consensus regarding subclassification criteria. LD type expresses the highly sensitive diagnostic marker S100 calcium-binding protein P (S100P), while SD type lacks sensitive markers. We identified osteopontin (OPN) as a highly sensitive marker for SD type. This study aimed to develop new subclassification criteria for LD-type and SD-type iCCA. We retrospectively investigated 74 patients with iCCA and subclassified them based on whole-section immunostaining of S100P and OPN. Of the 74 cases, 41 were subclassified as LD type, 32 as SD type, and one was indeterminate. Notably, all S100P-negative cases had OPN positivity. Seventy-three of the 74 cases (98.6%) were clearly and easily subclassified as LD or SD type using only these 2 markers. We also determined the value of immunohistochemistry in cases that were difficult to diagnose based on hematoxylin-eosin and Alcian blue-periodic acid-Schiff staining. Furthermore, we analyzed the clinicopathological characteristics and prognoses of these 2 subtypes. LD type was a poor prognostic factor on univariate analysis; it had significantly worse overall survival ( P = 0.007) and recurrence-free survival ( P < 0.001) than the SD type. In conclusion, we propose new subclassification criteria for iCCA based on immunostaining of S100P and OPN. These criteria may help pathologists to diagnose subtypes of iCCA, supporting future clinical trials and the development of medications for these 2 subtypes as distinct cancers.

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.