Liver & intrahepatic bile ducts

General

Staging-cholangiocarcinoma


Editor-in-Chief: Debra L. Zynger, M.D.
Raul S. Gonzalez, M.D.

Topic Completed: 10 December 2020

Minor changes: 24 June 2021

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PubMed Search: TNM staging[TIAB] intrahepatic bile duct tumors


Raul S. Gonzalez, M.D.
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Cite this page: Gonzalez RS. Staging-cholangiocarcinoma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/livertumortnmintrahepatic.html. Accessed December 8th, 2021.

Pathologic TNM staging of intrahepatic bile duct carcinomas, AJCC 8th edition
Definition / general
  • All intrahepatic bile duct carcinomas, including combined hepatocellular cholangiocarcinomas and poorly differentiated neuroendocrine carcinomas, are covered by this staging system
  • Not covered by this staging system are carcinomas of the perihilar or distal bile ducts
Essential features
ICD coding
  • C22.1: Intrahepatic bile duct carcinoma
Primary tumor (pT)
  • TX: Primary tumor cannot be assessed
  • T0: No evidence of primary tumor
  • Tis: Carcinoma in situ (intraductal tumor)
  • T1: Solitary tumor without vascular invasion
    • T1a: Solitary tumor ≤ 5 cm without vascular invasion
    • T1b: Solitary tumor > 5 cm without vascular invasion
  • T2: Solitary tumor with intrahepatic vascular invasion or multiple tumors, with or without vascular invasion
  • T3: Tumor perforating the visceral peritoneum
  • T4: Tumor involving local extrahepatic structures by direct invasion

Note:
  • Tumor growth patterns (mass forming versus periductal) are no longer part of staging criteria but should still be reported
Regional lymph nodes (pN)
  • NX: Regional lymph nodes cannot be assessed
  • N0: No regional lymph node metastasis
  • N1: Regional lymph node metastasis

Notes:
  • Regional lymph nodes depend on tumor site
  • For left sided lesions, regional nodes include inferior phrenic, hilar and gastrohepatic lymph nodes
  • For right sided lesions, regional nodes include hilar, periduodenal and peripancreatic lymph nodes
Distant metastasis (pM)
  • M0: No distant metastasis
  • M1: Distant metastasis
Prefixes
  • y: Preoperative radiotherapy or chemotherapy
  • r: Recurrent tumor stage
Stage grouping
Stage 0: Tis N0 M0
Stage IA: T1a N0 M0
Stage IB: T1b N0 M0
Stage IIA: T2 N0 M0
Stage IIIA: T3 N0 M0
Stage IIIB: T4 N0 M0
any T N1 M0
Stage IV: any T any N M1
Registry data collection variables
  • Presence of nontumoral hepatic parenchymal fibrosis / cirrhosis
  • Primary sclerosing cholangitis
  • Serum CA 19-9 level
  • Tumor growth pattern
Histologic grade
  • GX: Grade cannot be assessed
  • G1: Well differentiated
  • G2: Moderately differentiated
  • G3: Poorly differentiated
Histopathologic type
  • Intrahepatic cholangiocarcinoma
  • Combined hepatocellar cholangiocarcinoma
  • Carcinosarcoma
  • Intraductal papillary neoplasm with an associated invasive carcinoma
  • Mucinous cystic neoplasm with an associated invasive carcinoma
  • Bile duct cystadenocarcinoma
  • Neuroendocrine carcinoma, NOS
  • Large cell neuroendocrine carcinoma
  • Small cell neuroendocrine carcinoma
Board review style question #1
Which of the following is true regarding AJCC 8th edition staging of intrahepatic bile duct carcinomas?

  1. Combined hepatocellular cholangiocarcinomas are staged using different criteria
  2. Periductal invasion automatically characterizes a tumor as pT4
  3. Regional lymph nodes are different for left sided and right sided tumors
  4. Size cutoff of 5 cm is used to distinguish pT1 from pT2 tumors
Board review style answer #1
C. Regional lymph nodes are different for left sided and right sided tumors

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