Review
Copyright ©The Author(s) 2021.
World J Gastrointest Pathophysiol. Sep 22, 2021; 12(5): 84-105
Published online Sep 22, 2021. doi: 10.4291/wjgp.v12.i5.84
Table 1 Summary of anatomical, histomorphological, and molecular characteristics of cholangiocarcinoma subtypes
Anatomical classification
Histomorphological classification
Molecular specification (gene alterations)
iCCASmall intrahepatic bile ducts iCCA Mass forming tumors[17]IDH1/2, FGFR2, EPHA2, BAP1[14,19]
Large intrahepatic bile ducts iCCAMass forming, periductal, or intraductal mucinous tumors[17,18]EPHA2, BAP1, KRAS, TP53, GNAS, NRAS, MRAS, SMAD4[12,14,21]
eCCAPerihilar CCAIntraductal mucinous tumors[12,17]KRAS, TP53, GNAS, NRAS, MRAS, SMAD4, ARID1B, PRKACA, BRAF[14,19,24]
Distal CCAPeriductal mucinous tumors[12,17]KRAS, TP53, GNAS, NRAS, MRAS, SMAD4, ARID1B, PRKACB, BRAF[14,19,24]