New staging system and a registry for perihilar cholangiocarcinoma

Abstract

Perihilar cholangiocarcinoma is one of the most challenging diseases with poor overall survival. The major problem for anyone trying to convincingly compare studies among centers or over time is the lack of a reliable staging system. The most commonly used system is the Bismuth-Corlette classification of bile duct involvement, which, however, does not include crucial information such as vascular encasement and distant metastases. Other systems are rarely used because they do not provide several key pieces of information guiding therapy. Therefore, we have designed a new system reporting the size of the tumor, the extent of the disease in the biliary system, the involvement of the hepatic artery and portal vein, the involvement of lymph nodes, distant metastases, and the volume of the putative remnant liver after resection. The aim of this system is the standardization of the reporting of perihilar cholangiocarcinoma so that relevant information regarding resectability, indications for liver transplantation, and prognosis can be provided. With this tool, we have created a new registry enabling every center to prospectively enter data on their patients with hilar cholangiocarcinoma (www.cholangioca.org). The availability of such standardized and multicenter data will enable us to identify the critical criteria guiding therapy. (HEPATOLOGY 2011;53:1363-1371 C holangiocarcinoma (CCA) arises from the malignant transformation of the bile duct epithelium; it represents approximately 10% of all primary hepatobiliary cancers and accounts for approximately 2% of all malignancies.1,2 Several lines of evidence indicate that the incidence of CCA has increased over the past 3 decades.3,4 These tumors can develop anywhere along the biliary tree and represent a quite heterogeneous group with distinct patterns, epidemiologies, clinical presentations, and prognoses. The most commonly used classification of CCA has three groups based on the location along the biliary tree: intrahepatic cholangiocarcinoma (IHC); perihilar cholangiocarcinoma (PHC), which is also called a Klatskin tumor 5 ; and distal CCA. The IHC type accounts for less than 10% of the total cases, whereas the PHC type represents about two-thirds of the cases, and distal CCA represents about a quarter of the cases.6 PHC can be defined as tumors that involve or are in close vicinity to the bile duct confluence. We suggest a definition of PHC, which includes tumors above the junction of the cystic duct up to and including the second biliary branches of the right and left bile ducts.The only chance of a cure for this type of cancer is complete surgical resection of the tumor and perhaps liver transplantation in highly select cases. Most of these cancers have a dismal prognosis, and the current 5-year

Cholangiocarcinoma: current knowledge and future perspectives consensus statement from the European Network for the Study of Cholangiocarcinoma (ENS-CCA)

“…By specifically focusing on predicting resectability and outcomes, the Memorial Sloan Kettering Cancer Center group proposed a staging system that classifies pCCA on the basis of the local tumour extension, site of bile duct involvement, portal vein invasion and hepatic lobar atrophy, although the size of the remnant liver is not specified 232 . In 2011, the Mayo Clinic proposed a staging system comprising the tumour size, the extent of the disease in the biliary system, the involvement of the hepatic artery and portal vein, the involvement of lymph nodes, distant metastasis and the volume of the putative remnant liver after resection 233 . Although complex, this classification has the merit of finely defining surgical options, standardizing prospective reporting of pCCA and discriminating between prognostic classes.…”

Risk factors and classifications of hilar cholangiocarcinoma

“…For this reason, an international working group was constituted with the aim to design a new staging system and registry for these tumors. The results of this project were published in 2011, proposing a new classification for Klatskin tumors using some parameters from previous staging systems [24] ( Table 3). The BC classification is kept for the assessment of the bile duct (which is labeled ''B'' for bile duct or Bismuth); the letters ''a'' and ''b'' are omitted and are replaced by ''R'' (for right hepatic duct) and ''L'' (for left hepatic duct; Figure 3A).…”

Comprehensive management of cholangiocarcinoma: Part I. Diagnosis

“…A new system has been proposed recently that was designed to assist with more accurate pre-or postoperative staging of the PCC. 28 This staging system, besides ductal involvement, incorporates more parameters in PCC staging: 1) tumor size (< 1 to > 3 cm in size), 2) tumor type (sclerosing, MF, mixed and polypoid or IG), 3) involvement of portal vein (PV) or hepatic artery (HA), 4) involvement of regional and distant LN, 5) remnant liver volume after resection, and 6) underlying liver disease such as fibrosis and PSC. More recently, the BC, MSKCC and TNM systems were compared in patients with PCC and it was found that TNM and MSKCC systems, tumor differentiation, LN status, distant metastases and R status were significantly correlated with survival, whereas BC classification, age, gender, jaundice, and upper abdominal pain were not.…”