NCI Definition: A carcinoma that arises from the intrahepatic bile duct epithelium in any site of the intrahepatic biliary tree. Grossly, the malignant lesions are solid, nodular, and grayish. Morphologically, the vast majority of cases are adenocarcinomas. Signs and symptoms include malaise, weight loss, right upper quadrant abdominal pain, and night sweats. Early detection is difficult and the prognosis is generally poor. [1]

Intrahepatic cholangiocarcinomas most frequently harbor alterations in IDH1, TP53, BAP1, FGFR2, and ARID1A [2].

Most Commonly Altered Genes in Intrahepatic Cholangiocarcinoma

IDH1 Mutation, IDH1 Codon 132 Missense, IDH1 R132C, TP53 Mutation, and FGFR2 Fusion are the most common alterations in intrahepatic cholangiocarcinoma [2].

Top Alterations in Intrahepatic Cholangiocarcinoma

Significant Genes in Intrahepatic Cholangiocarcinoma


FGF19 +

FGF9 +





IDH1 +

Disease Details

ICC, Intrahepatic Carcinoma of Bile Duct, Intrahepatic Cholangiocellular Carcinoma, CHOLANGIOCARCINOMA, INTRAHEPATIC, MALIGNANT, Intrahepatic Carcinoma of the Bile Duct, Intrahepatic Bile Duct Carcinoma, Liver cholangiocarcinoma
Liver and Intrahepatic Bile Duct Carcinoma
Signet Ring Cell Intrahepatic Cholangiocarcinoma, Sarcomatoid Intrahepatic Cholangiocarcinoma, Mucin-Producing Intrahepatic Cholangiocarcinoma, Cholangiolocellular Carcinoma, and Perihilar Intrahepatic Cholangiocarcinoma
OncoTree Name
Intrahepatic Cholangiocarcinoma
OncoTree Code


1. National Cancer Institute. NCI Thesaurus Version 18.11d. https://ncit.nci.nih.gov/ncitbrowser/ [2018-08-28]. [2018-09-21].

2. The AACR Project GENIE Consortium. AACR Project GENIE: powering precision medicine through an international consortium. Cancer Discovery. 2017;7(8):818-831. Dataset Version 8. This dataset does not represent the totality of the genetic landscape; see paper for more information.

3. All assertions and clinical trial landscape data are curated from primary sources. You can read more about the curation process here.