Perihilar cholangiocarcinoma (staging)

Last revised by Francis Deng on 20 Dec 2020

Perihilar cholangiocarcinoma staging is, for prognostication, most commonly conducted using the TNM staging classification of the American Joint Committee on Cancer (AJCC)/Union for International Cancer Control (UICC). As of 2018, the staging criteria are in their 8th edition and reflected below 1. These criteria apply to hilar/perihilar cholangiocarcinomas (Klatskin tumor), while cancers of the intrahepatic and distal bile ducts are staged differently.

  • TX: primary tumor cannot be assessed 
  • T0: no evidence of primary tumor
  • Tis: carcinoma in situ/high-grade dysplasia
  • T1: tumor confined to the bile duct, with extension up to the muscle layer or fibrous tissue
  • T2
    • T2a: tumor invades beyond the wall of the bile duct to surrounding adipose tissue
    • T2b: tumor invades adjacent hepatic parenchyma
  • T3: tumor invades unilateral branches of the portal vein or hepatic artery
  • T4
    • tumor invades the main portal vein or its branches bilaterally, or
    • unilateral second-order biliary radicals with contralateral portal vein or hepatic artery involvement

The regional lymph nodes are hilar, cystic duct, common bile duct, hepatic artery, posterior pancreatoduodenal, and portal vein lymph nodes.

  • NX: regional lymph nodes cannot be assessed
  • N0: no regional lymph node metastasis
  • N1: 1-3 positive regional lymph nodes
  • N2: 4 or more positive regional lymph nodes
  • cM0: no evidence of metastases
  • cM1: distant metastasis
  • pM1: distant metastasis, microscopically confirmed
  • stage 0
    • Tis, N0, M0
  • stage I
    • T1, N0, M0
  • stage II
    • [T2a, T2b], N0, M0
  • stage IIIA
    • T3, N0, M0 
  • stage IIIB
    • T4, N0, M0
  • stage IIIC
    • [Any T], N1, M0
  • stage IVA
    • [Any T], N2, M0
  • stage IVB
    • [Any T], [Any N], M1

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