Missing content? – Request curation!
Request curation for specific Genes, variants, or PubMed publications.
Have questions, comments or suggestions? - Let us know!
Email us at : ckbsupport@jax.org
ID | DOID:4928 |
Name | intrahepatic cholangiocarcinoma |
Definition | A cholangiocarcinoma that arises from the intrahepatic bile duct epithelium in any site of the intrahepatic biliary tree. |
Source | DiseaseOntology.org |
Alt Ids | |
Path | disease disease of cellular proliferation cancer organ system cancer gastrointestinal system cancer hepatobiliary system cancer biliary tract cancer bile duct cancer bile duct carcinoma bile duct adenocarcinoma cholangiocarcinoma intrahepatic cholangiocarcinoma |
Molecular Profile | Therapy | Indication/Tumor Type | Response Type | Profile Response Detail |
---|---|---|---|---|
FGFR2 fusion | Derazantinib | intrahepatic cholangiocarcinoma | sensitive | detail... |
IDH1 R132S | Dasatinib | intrahepatic cholangiocarcinoma | sensitive | detail... |
IDH1 R132S | Saracatinib | intrahepatic cholangiocarcinoma | sensitive | detail... |
IDH1 R132C | Saracatinib | intrahepatic cholangiocarcinoma | sensitive | detail... |
IDH1 R132C | Dasatinib | intrahepatic cholangiocarcinoma | sensitive | detail... |
IDH1 R132C SRC T341I | Dasatinib | intrahepatic cholangiocarcinoma | resistant | detail... |
FGFR2 F276C | Infigratinib | intrahepatic cholangiocarcinoma | predicted - sensitive | detail... |
FGFR2 - SORBS1 FGFR2 K660M FGFR2 K715R | Infigratinib | intrahepatic cholangiocarcinoma | resistant | detail... |
FGFR2 - SORBS1 FGFR2 K660M FGFR2 K715R | Futibatinib | intrahepatic cholangiocarcinoma | sensitive | detail... |
FGFR2 - SORBS1 | Infigratinib | intrahepatic cholangiocarcinoma | sensitive | detail... |
FGFR2 - SORBS1 FGFR2 V565F | Futibatinib | intrahepatic cholangiocarcinoma | resistant | detail... |
FGFR2 - ZMYM4 | Infigratinib | intrahepatic cholangiocarcinoma | sensitive | detail... |
FGFR2 - ZMYM4 FGFR2 N550H FGFR2 N550K FGFR2 V565F FGFR2 E566A FGFR2 K660M | Infigratinib | intrahepatic cholangiocarcinoma | resistant | detail... |
FGFR2 - ZMYM4 FGFR2 N550H FGFR2 N550K FGFR2 V565F FGFR2 E566A FGFR2 K660M | Futibatinib | intrahepatic cholangiocarcinoma | sensitive | detail... |
FGFR2 - ZMYM4 FGFR2 V563L | Futibatinib | intrahepatic cholangiocarcinoma | resistant | detail... |
FGFR2 - INA | Debio 1347 | intrahepatic cholangiocarcinoma | sensitive | detail... |
FGFR2 - INA FGFR2 M538I FGFR2 N550H FGFR2 N550T FGFR2 L618V FGFR2 H683L | Debio 1347 | intrahepatic cholangiocarcinoma | resistant | detail... |
FGFR2 - INA FGFR2 M538I FGFR2 N550H FGFR2 N550T FGFR2 L618V FGFR2 H683L | Futibatinib | intrahepatic cholangiocarcinoma | sensitive | detail... |
FGFR2 - INA FGFR2 V565L FGFR2 E566A | Futibatinib | intrahepatic cholangiocarcinoma | resistant | detail... |
FGFR2 - NRAP | Infigratinib | intrahepatic cholangiocarcinoma | sensitive | detail... |
FGFR2 - NRAP | Futibatinib | intrahepatic cholangiocarcinoma | sensitive | detail... |
FGFR2 - NRAP FGFR2 N550K | Futibatinib | intrahepatic cholangiocarcinoma | resistant | detail... |
FGFR2 - KIAA1217 | Futibatinib | intrahepatic cholangiocarcinoma | sensitive | detail... |
FGFR2 fusion FGFR2 M538I | Infigratinib | intrahepatic cholangiocarcinoma | decreased response | detail... |
FGFR2 fusion FGFR2 N550H | Infigratinib | intrahepatic cholangiocarcinoma | decreased response | detail... |
FGFR2 fusion FGFR2 E566A | Infigratinib | intrahepatic cholangiocarcinoma | decreased response | detail... |
FGFR2 fusion FGFR2 H683L | Infigratinib | intrahepatic cholangiocarcinoma | decreased response | detail... |
FGFR2 fusion FGFR2 N550K | Infigratinib | intrahepatic cholangiocarcinoma | resistant | detail... |
FGFR2 fusion FGFR2 V565F | Infigratinib | intrahepatic cholangiocarcinoma | resistant | detail... |
FGFR2 fusion FGFR2 L618V | Infigratinib | intrahepatic cholangiocarcinoma | resistant | detail... |
FGFR2 fusion FGFR2 K660M | Infigratinib | intrahepatic cholangiocarcinoma | resistant | detail... |
FGFR2 fusion FGFR2 K715R | Infigratinib | intrahepatic cholangiocarcinoma | sensitive | detail... |
FGFR2 fusion FGFR2 K715R | Debio 1347 | intrahepatic cholangiocarcinoma | sensitive | detail... |
FGFR2 fusion FGFR2 M538I | Debio 1347 | intrahepatic cholangiocarcinoma | decreased response | detail... |
FGFR2 fusion FGFR2 N550H | Debio 1347 | intrahepatic cholangiocarcinoma | decreased response | detail... |
FGFR2 fusion FGFR2 V565F | Debio 1347 | intrahepatic cholangiocarcinoma | decreased response | detail... |
FGFR2 fusion FGFR2 E566A | Debio 1347 | intrahepatic cholangiocarcinoma | decreased response | detail... |
FGFR2 fusion FGFR2 H683L | Debio 1347 | intrahepatic cholangiocarcinoma | decreased response | detail... |
FGFR2 fusion FGFR2 N550K | Debio 1347 | intrahepatic cholangiocarcinoma | resistant | detail... |
FGFR2 fusion FGFR2 L618V | Debio 1347 | intrahepatic cholangiocarcinoma | resistant | detail... |
FGFR2 fusion FGFR2 K660M | Debio 1347 | intrahepatic cholangiocarcinoma | resistant | detail... |
FGFR2 fusion FGFR2 V565F | Futibatinib | intrahepatic cholangiocarcinoma | resistant | detail... |
FGFR2 fusion FGFR2 K715R | Futibatinib | intrahepatic cholangiocarcinoma | sensitive | detail... |
FGFR2 fusion FGFR2 M538I | Futibatinib | intrahepatic cholangiocarcinoma | sensitive | detail... |
FGFR2 fusion FGFR2 N550H | Futibatinib | intrahepatic cholangiocarcinoma | sensitive | detail... |
FGFR2 fusion FGFR2 N550K | Futibatinib | intrahepatic cholangiocarcinoma | sensitive | detail... |
FGFR2 fusion FGFR2 E566A | Futibatinib | intrahepatic cholangiocarcinoma | sensitive | detail... |
FGFR2 fusion FGFR2 L618V | Futibatinib | intrahepatic cholangiocarcinoma | sensitive | detail... |
FGFR2 fusion FGFR2 K660M | Futibatinib | intrahepatic cholangiocarcinoma | sensitive | detail... |
FGFR2 fusion FGFR2 H683L | Futibatinib | intrahepatic cholangiocarcinoma | sensitive | detail... |
NTRK1 fusion | Larotrectinib | intrahepatic cholangiocarcinoma | sensitive | detail... |
NTRK2 fusion | Larotrectinib | intrahepatic cholangiocarcinoma | sensitive | detail... |
NTRK1 fusion | Entrectinib | intrahepatic cholangiocarcinoma | sensitive | detail... |
NTRK2 fusion | Entrectinib | intrahepatic cholangiocarcinoma | sensitive | detail... |
FGFR2 - BICC1 | Derazantinib | intrahepatic cholangiocarcinoma | predicted - sensitive | detail... |
FGFR2 - KIAA1217 | Derazantinib | intrahepatic cholangiocarcinoma | predicted - sensitive | detail... |
FGFR2 - SH3GLB1 | Derazantinib | intrahepatic cholangiocarcinoma | predicted - sensitive | detail... |
FGFR2 - CCAR2 | Derazantinib | intrahepatic cholangiocarcinoma | predicted - sensitive | detail... |
FGFR2 - AHCYL1 | Derazantinib | intrahepatic cholangiocarcinoma | predicted - sensitive | detail... |
FGFR2 - BFSP2 | Derazantinib | intrahepatic cholangiocarcinoma | predicted - sensitive | detail... |
FGFR2 - CCDC6 | Derazantinib | intrahepatic cholangiocarcinoma | predicted - sensitive | detail... |
FGFR2 - TACC2 | Derazantinib | intrahepatic cholangiocarcinoma | predicted - sensitive | detail... |
FGFR2 - CIT | Derazantinib | intrahepatic cholangiocarcinoma | predicted - resistant | detail... |
FGFR2 - TACC1 | Derazantinib | intrahepatic cholangiocarcinoma | predicted - resistant | detail... |
FGFR2 H167_N173del | Debio 1347 | intrahepatic cholangiocarcinoma | predicted - sensitive | detail... |
FGFR2 H167_N173del FGFR2 L617F | Debio 1347 | intrahepatic cholangiocarcinoma | predicted - resistant | detail... |
RET fusion | Pralsetinib | intrahepatic cholangiocarcinoma | sensitive | detail... |
FGFR2 Y376C | AZD4547 | intrahepatic cholangiocarcinoma | predicted - sensitive | detail... |
FGFR2 fusion | Futibatinib | intrahepatic cholangiocarcinoma | predicted - sensitive | detail... |
FGFR2 rearrange | Futibatinib | intrahepatic cholangiocarcinoma | predicted - sensitive | detail... |
FGFR2 - BICC1 | Futibatinib | intrahepatic cholangiocarcinoma | predicted - sensitive | detail... |
BRAF V600E | Trametinib | intrahepatic cholangiocarcinoma | predicted - sensitive | detail... |
FGFR2 - SORBS1 | Futibatinib | intrahepatic cholangiocarcinoma | predicted - sensitive | detail... |
FGFR1 N546D | Futibatinib | intrahepatic cholangiocarcinoma | predicted - sensitive | detail... |
FGFR2 - PHGDH | Debio 1347 | intrahepatic cholangiocarcinoma | predicted - sensitive | detail... |
FGFR2 - PHGDH | Futibatinib | intrahepatic cholangiocarcinoma | predicted - sensitive | detail... |
FGFR2 H167_N173del | Futibatinib | intrahepatic cholangiocarcinoma | predicted - sensitive | detail... |
FGFR2 I288_E295delinsT | Debio 1347 | intrahepatic cholangiocarcinoma | predicted - sensitive | detail... |
FGFR2 H167_N173del FGFR2 L618F | Debio 1347 | intrahepatic cholangiocarcinoma | predicted - resistant | detail... |
FGFR2 H167_N173del FGFR2 L618F | Futibatinib | intrahepatic cholangiocarcinoma | predicted - sensitive | detail... |
BRAF V600E | Dabrafenib + Trametinib | intrahepatic cholangiocarcinoma | predicted - sensitive | detail... |
BRAF L597Q FGFR2 H167_N173del FGFR2 L618F | Futibatinib | intrahepatic cholangiocarcinoma | predicted - resistant | detail... |
BRAF L597Q FGFR2 H167_N173del FGFR2 L618F | LY3214996 | intrahepatic cholangiocarcinoma | no benefit | detail... |
BRAF L597Q FGFR2 H167_N173del FGFR2 N550K FGFR2 L618F NRAS Q61K | LY3214996 | intrahepatic cholangiocarcinoma | predicted - resistant | detail... |
FGFR2 - PPHLN1 BRAF V600E | Infigratinib | intrahepatic cholangiocarcinoma | predicted - resistant | detail... |
FGFR2 - PPHLN1 NRAS G12V | Infigratinib | intrahepatic cholangiocarcinoma | predicted - resistant | detail... |
FGFR2 - CCDC6 | Trametinib | intrahepatic cholangiocarcinoma | sensitive | detail... |
FGFR2 - CCDC6 | Infigratinib + Trametinib | intrahepatic cholangiocarcinoma | sensitive | detail... |
FGFR2 - CCDC6 | Infigratinib | intrahepatic cholangiocarcinoma | sensitive | detail... |
FGFR2 - TACC3 | Infigratinib | intrahepatic cholangiocarcinoma | sensitive | detail... |
FGFR2 - TACC3 | Trametinib | intrahepatic cholangiocarcinoma | sensitive | detail... |
FGFR2 - TACC3 | Infigratinib + Trametinib | intrahepatic cholangiocarcinoma | sensitive | detail... |
FGFR2 - TACC3 FGFR2 V565F | Trametinib | intrahepatic cholangiocarcinoma | sensitive | detail... |
FGFR2 - TACC3 FGFR2 V565F | Infigratinib | intrahepatic cholangiocarcinoma | resistant | detail... |
FGFR2 - BICC1 | Infigratinib + Trametinib | intrahepatic cholangiocarcinoma | sensitive | detail... |
FGFR2 - BICC1 | Trametinib | intrahepatic cholangiocarcinoma | no benefit | detail... |
FGFR2 - BICC1 | Infigratinib | intrahepatic cholangiocarcinoma | sensitive | detail... |
FGFR2 fusion FGFR2 V564F | RLY-4008 | intrahepatic cholangiocarcinoma | predicted - sensitive | detail... |
MLH1 negative | Dostarlimab-gxly | intrahepatic cholangiocarcinoma | sensitive | detail... |
MSH6 negative | Dostarlimab-gxly | intrahepatic cholangiocarcinoma | sensitive | detail... |
MLH1 negative | Pembrolizumab | intrahepatic cholangiocarcinoma | sensitive | detail... |
MSH6 negative | Pembrolizumab | intrahepatic cholangiocarcinoma | sensitive | detail... |
FGFR2 F276C | Pazopanib | intrahepatic cholangiocarcinoma | predicted - sensitive | detail... |
FGFR2 T370_A371delinsS | Lenvatinib | intrahepatic cholangiocarcinoma | predicted - sensitive | detail... |
IDH1 act mut | Olutasidenib | intrahepatic cholangiocarcinoma | predicted - sensitive | detail... |
FGFR2 - CCDC6 | Futibatinib | intrahepatic cholangiocarcinoma | predicted - sensitive | detail... |
FGFR2 - TTC28 | Futibatinib | intrahepatic cholangiocarcinoma | predicted - sensitive | detail... |
FGFR2 - TNS1 | Futibatinib | intrahepatic cholangiocarcinoma | predicted - sensitive | detail... |
FGFR2 - WAC | Futibatinib | intrahepatic cholangiocarcinoma | predicted - sensitive | detail... |
FGFR2 W290C | Futibatinib | intrahepatic cholangiocarcinoma | predicted - sensitive | detail... |
FGFR2 C383R | Futibatinib | intrahepatic cholangiocarcinoma | predicted - sensitive | detail... |
FGFR1 M563T | Futibatinib | intrahepatic cholangiocarcinoma | predicted - sensitive | detail... |
Clinical Trial | Phase | Therapies | Title | Recruitment Status | Covered Countries | Other Countries |
---|---|---|---|---|---|---|
NCT02428855 | Phase II | Dasatinib | Phase II Trial of Dasatinib in Patients With Isocitrate Dehydrogenase (IDH)-Mutant Advanced Intrahepatic Cholangiocarcinoma | Completed | USA | 0 |
NCT02632305 | Phase II | Cisplatin + Gemcitabine + Nab-paclitaxel | A Study to See the Effects That a New Combination of the Three Drugs, Nab-paclitaxel, Gemcitabine, and Cisplatin Has on Biliary Tract Cancer (AX-CSARC) | Unknown status | CAN | 0 |
NCT02834013 | Phase 0 | Ipilimumab + Nivolumab | Nivolumab and Ipilimumab in Treating Patients With Rare Tumors | Recruiting | USA | 1 |
NCT02834780 | Phase I | H3B-6527 | Phase 1 Study to Evaluate the Safety, Pharmacokinetics and Pharmacodynamics of H3B-6527 in Subjects With Advanced Hepatocellular Carcinoma | Completed | USA | ITA | FRA | ESP | CAN | BEL | 5 |
NCT03230318 | Phase II | Derazantinib | Derazantinib in Subjects With FGFR2 Gene Fusion-, Mutation- or Amplification- Positive Inoperable or Advanced Intrahepatic Cholangiocarcinoma (FIDES-01) | Active, not recruiting | USA | ITA | FRA | ESP | DEU | CAN | BEL | 4 |
NCT03684811 | Phase Ib/II | Cisplatin + Gemcitabine + Olutasidenib Azacitidine + Olutasidenib Olutasidenib Nivolumab + Olutasidenib | A Study of FT-2102 in Patients With Advanced Solid Tumors and Gliomas With an IDH1 Mutation | Completed | USA | FRA | ESP | 3 |
NCT03781934 | Phase Ib/II | MIV-818 | A Study to Evaluate MIV-818 in Patients With Liver Cancer Manifestations | Recruiting | BEL | 1 |
NCT04003636 | Phase III | Cisplatin + Gemcitabine Cisplatin + Gemcitabine + Pembrolizumab | Pembrolizumab (MK-3475) Plus Gemcitabine/Cisplatin Versus Placebo Plus Gemcitabine/Cisplatin for First-Line Advanced and/or Unresectable Biliary Tract Carcinoma (BTC) (MK-3475-966/KEYNOTE-966) (KEYNOTE-966) | Active, not recruiting | USA | ITA | FRA | ESP | DEU | CAN | BEL | 17 |
NCT04057365 | Phase II | DKN-01 + Nivolumab | A Single Arm Phase II Study of the Combination of DKN-01 and Nivolumab in Previously Treated Patients With Advanced Biliary Tract Cancer (BTC) | Recruiting | USA | 0 |
NCT04093362 | Phase III | Futibatinib Cisplatin + Gemcitabine | Futibatinib Vs Gemcitabine-Cisplatin Chemotherapy as 1st-Line Treatment of Patients With Advanced Cholangiocarcinoma (CCA) Harboring FGFR2 Gene Rearrangements (FOENIX-CCA3) | Recruiting | USA | ITA | FRA | ESP | DEU | BEL | 14 |
NCT04175912 | Phase II | Carboplatin + MLN4924 + Paclitaxel MLN4924 | Testing the Combination of Pevonedistat With Chemotherapy for Bile Duct Cancer of the Liver | Suspended | USA | 0 |
NCT04203160 | Phase Ib/II | Cisplatin + Gemcitabine Cisplatin + CPI-613 + Gemcitabine | Gemcitabine and Cisplatin With or Without CPI-613 as First Line Therapy for Patients With Advanced Unresectable Biliary Tract Cancer (BilT-04) | Recruiting | USA | 0 |
NCT04301778 | Phase II | Durvalumab + SNDX-6352 | Durvalumab in Combination With a CSF-1R Inhibitor (SNDX-6532) Following Chemo or Radio-Embolization for Patients With Intrahepatic Cholangiocarcinoma | Recruiting | USA | 0 |
NCT04306367 | Phase II | Olaparib + Pembrolizumab | Study of Pembrolizumab and Olaparib in Bile Duct Cancer | Recruiting | USA | 0 |
NCT04466891 | Phase II | ZW25 | A Study of ZW25 (Zanidatamab) in Subjects With Advanced or Metastatic HER2-Amplified Biliary Tract Cancers | Active, not recruiting | USA | ITA | FRA | ESP | CAN | 4 |
NCT04704154 | Phase II | Nivolumab + Regorafenib | A Trial to Learn Whether Regorafenib in Combination With Nivolumab Can Improve Tumor Responses and How Safe it is for Participants With Solid Tumors | Active, not recruiting | USA | ITA | FRA | BEL | 4 |
NCT04708067 | Phase I | M7824 | Hypofractionated Radiation Therapy and Bintrafusp Alfa for the Treatment of Advanced Intrahepatic Cholangiocarcinoma | Recruiting | USA | 0 |
NCT04907851 | Phase II | Denosumab + RXC004 | A Study to Assess RXC004 Efficacy in Advanced Solid Tumours After Progression on Standard of Care (SoC) Therapy (PORCUPINE2) | Recruiting | 2 | |
NCT04941287 | Phase II | Atezolizumab + Varlilumab Atezolizumab + Cobimetinib + Varlilumab | Testing A New Combination of Anti-cancer Immune Therapies, Atezolizumab and CDX-1127 (Varlilumab) With or Without the Addition of A Third Anti-cancer Drug, Cobimetinib, for Advanced-Stage Biliary Tract Cancer | Recruiting | USA | 0 |
NCT04969887 | Phase II | Ipilimumab + Nivolumab | Combination Immunotherapy in Rare Cancers Under InvesTigation (MOST-CIRCUIT) | Recruiting | 1 | |
NCT04976634 | Phase II | Belzutifan + Lenvatinib + Pembrolizumab | Pembrolizumab Plus Lenvatinib in Combination With Belzutifan in Solid Tumors (MK-6482-016) | Recruiting | USA | FRA | ESP | BEL | 5 |
NCT04989218 | Phase Ib/II | Cisplatin + Durvalumab + Gemcitabine + Tremelimumab | Durvalumab and Tremelimumab With Platinum-based Chemotherapy in Intrahepatic Cholangiocarcinoma (ICC) (ICC) | Recruiting | USA | 0 |
NCT05007106 | Phase II | MK-7684A + Paclitaxel Cisplatin + Fluorouracil + MK-7684A Pembrolizumab Lenvatinib + MK-7684A MK-7684A | MK-7684A With or Without Other Anticancer Therapies in Participants With Selected Solid Tumors (MK-7684A-005) | Recruiting | USA | ITA | FRA | ESP | DEU | CAN | 9 |
NCT05174650 | Phase II | Atezolizumab + Derazantinib | Treatment of Atezolizumab and Derazantinib in Patients With Advanced iCCA With FGFR2 Fusions/Rearrangements | Recruiting | DEU | 0 |
NCT05220722 | Phase Ib/II | Pembrolizumab + SD-101 Ipilimumab + Nivolumab + SD-101 | Pressure Enabled Delivery of SD-101 With Checkpoint Blockade for Primary Liver Tumors | Recruiting | USA | 0 |
NCT05221320 | Phase II | Hydroxychloroquine + Ulixertinib | Trial of Ulixertinib in Combination With Hydroxychloroquine in Patients With Advanced Gastrointestinal (GI) Malignancies | Recruiting | USA | 0 |
NCT05239169 | Phase II | Capecitabine + Durvalumab + Tremelimumab Durvalumab + Tremelimumab | Immunotherapy With Durva and Treme With or Without Capecitabine in Adjuvant Treatment for Biliary Tract Cancer | Recruiting | DEU | 0 |
NCT05286814 | Phase II | Fluorouracil + Leucovorin + NHS-IL12 + Oxaliplatin Fluorouracil + Irinotecan + Leucovorin + NHS-IL12 | M9241 in Combination With Hepatic Artery Infusion Pump (HAIP) and Systemic Therapy for Subjects With Metastatic Colorectal Cancer or Intrahepatic Cholangiocarcinoma | Not yet recruiting | USA | 0 |