Subtype
Malignant Biliary Tract Neoplasm
CI-CAN-00003139Explore in graph →
Loading cancer entity…
Subtype
CI-CAN-00003139Explore in graph →
Variants & evidence
66 evidence items mapped to this entity or its descendants, grouped by molecular profile, then therapy. 50 items per page.
| Therapy | Cancer | Type | Level | Direction · significance | Rating (1–5) | Status | Evidence | Source |
|---|---|---|---|---|---|---|---|---|
| NRG1 Fusion1 | ||||||||
| Afatinib | Cholangiocarcinoma | Predictive | C | Supports Sensitivity Response | 3 | accepted | EID5858In a single patient with presumed cholangiocarcinoma a novel ATP1B1-NRG1 gene fusion was detected. Integrative genome analysis was used to assess the potential functional significance of the gene fusi… (full text at CIViC) PMID 28950338 · Jones et al., 2017 · Open in CIViC | civic |
| BRAF V6001 | ||||||||
| Vemurafenib | Cholangiocarcinoma | Predictive | B | Supports Sensitivity Response | 4 | accepted | EID5905Eight patients with BRAF V600 (7 with V600E, 1 with V600 unknown) mutated cholangiocarcinoma received vemurafenib. Only one patients showed partial response and duration of response of this patient wa… (full text at CIViC) PMID 26287849 · Hyman et al., 2015 · Open in CIViC | civic |
| BRAF V600E6 | ||||||||
| Dabrafenib + TrametinibCombination | Malignant Biliary Tract NeoplasmALIAS | Predictive | B | Supports Sensitivity Response | 3 | accepted | EID7264In a phase II trial, 33 patients with advanced or metastatic biliary tract cancer (BTC) received dabrafenib (D) and trametinib (T) in a second or higher line therapeutic context. Of the 33 patients, 3… (full text at CIViC) | civic |
| 〃 | Cholangiocarcinoma | Predictive | B | Supports Sensitivity Response | 3 | submitted | EID7453In NCI-MATCH trial, patients with advanced solid tumors, lymphoma or multiple myeloma harboring BRAF V600E mutation were treated with dabrafenib and trametinib. Patients with melanoma, thyroid carcino… (full text at CIViC) | civic |
| 〃 | Cholangiocarcinoma | Predictive | C | Supports Sensitivity Response | 3 | accepted | ||
| BRCA1 Mutation + BRCA2 MutationBRCA2BRCA11 | ||||||||
| Olaparib | Malignant Biliary Tract NeoplasmALIAS | Predictive | B | Supports Sensitivity Response | 4 | accepted | EID12764In the biliary tract carcinoma cohort of the phase II TAPUR trial (NCT02693535), patients with BRCA1/2 alterations treated with olaparib (18 evaluable) had 2 PR (BRCA1 loss n = 1, BRCA2 mutation n = 1… (full text at CIViC) PMID 41100773 · Al Baghdadi et al., 2025 · Open in CIViC | civic |
| CDKN2A Loss + CDKN2A Mutation1 | ||||||||
| Palbociclib | Malignant Biliary Tract NeoplasmALIAS | Predictive | B | Does Not Support Sensitivity Response | 3 | accepted | EID11654A phase II clinical study conducted in 10 patients with biliary cancer with CDKN2A loss or mutation, that were treated with palbociclib. No clinical activity demonstrated in patients with CDKN2A mutat… (full text at CIViC) PMID 35100714 · Al Baghdadi et al., 2019 · Open in CIViC | civic |
| CDKN2A Mutation1 | ||||||||
| Palbociclib | Cholangiocarcinoma | Predictive | B | Does Not Support Sensitivity Response | 4 | accepted | EID7300In a phase 2 trial, patients with gallbladder cancer or bile duct cancer with CDKN2A loss or mutation were received palbociclib. Among 10 patients, no overall response or stable disease at 16 weeks we… (full text at CIViC) | civic |
| EGFR Mutation1 | ||||||||
| (prognostic) | Cholangiocarcinoma | Prognostic | B | Supports Poor Outcome | 2 | submitted | EID10359In a study of 137 biliary tract carcinomas, 13 patients were identified to have EGFR variants in exons 18-21 (no other exons were evaluated). EGFR variants were associated with worse survival in univa… (full text at CIViC) PMID 24372748 · Chang et al., 2014 · Open in CIViC | civic |
| EGFR T790M1 | ||||||||
| (prognostic) | Cholangiocarcinoma | Prognostic | B | Supports Better Outcome | 1 | submitted | EID10358In a Thai cohort of 81 patients with cholangiocarcinoma, 17 patients harbored EGFR variants, 9 of which were T790M. EGFR T790M variants were associated with longer overall survival than patients with … (full text at CIViC) PMID 29066915 · Trachu et al., 2017 · Open in CIViC | civic |
| ERBB2 Amplification2 | ||||||||
| Pertuzumab + TrastuzumabCombination | Malignant Biliary Tract NeoplasmALIAS | Predictive | B | Supports Sensitivity Response | 2 | accepted | EID5983The phase 2a MyPathway study assigned patients with HER2, EGFR, BRAF or SHH alterations to treatment with pertuzumab plus trastuzumab, erlotinib, vemurafenib, or vismodegib, respectively. Two of seven… (full text at CIViC) PMID 29320312 · Hainsworth et al., 2018 · Open in CIViC | civic |
| Pertuzumab + TrastuzumabCombination | Cholangiocarcinoma | Predictive | C | Supports Sensitivity Response | 3 | accepted | EID7638A patient with cholangiocarcinoma harboring with ERBB2 amplification (3+) detected by circulating tumor DNA testing and confirmed by tissue-based testing was treated with trastuzumab and pertuzumab. T… (full text at CIViC) PMID 31453370 · Yarlagadda et al., 2019 · Open in CIViC | civic |
| ERBB2 G292R1 | ||||||||
| (oncogenic) | Gallbladder Carcinoma | Oncogenic | D | Supports Oncogenicity | 2 | submitted | EID12117The ERBB2 G292R variant was observed in 1/57 normal/tumor pairs of human gallbladder carcinoma profiled by next-generation sequencing. Human gallbladder cancer cell lines (GBC-SD and NOZ) were transie… (full text at CIViC) PMID 24997986 · Li et al., 2014 · Open in CIViC | civic |
| ERRFI1 E384*2 | ||||||||
| Erlotinib | Cholangiocarcinoma | Predictive | C | Supports Sensitivity Response | 3 | rejected | EID5869A patient with cholangiocarcinoma who has initially undergone surgical resection progressed on gemcitabine and cisplatin. He subsequently received pegylated hyaluroniade (PEGPH20) in an investigationa… (full text at CIViC) PMID 24550739 · Borad et al., 2014 · Open in CIViC | civic |
| 〃 | Cholangiocarcinoma | Predictive | C | Supports Sensitivity Response | 2 | accepted | EID1724An ERRFI1 (E384X) was detected in a patient with metastatic, recurrent/refractory cholangiocarcinoma (allelic fraction 11%) without any mutations or amplifications in other EGFR signaling members such… (full text at CIViC) PMID 24550739 · Borad et al., 2014 · Open in CIViC | civic |
| ACOT11 Fusion1 | ||||||||
| Erdafitinib | Intrahepatic Cholangiocarcinoma | Predictive | C | Supports Sensitivity Response | 3 | accepted | EID13163In the phase II NCI-MATCH EAY131-K2 trial, a patient with intrahepatic cholangiocarcinoma harboring an FGFR2-ACOT11 fusion achieved a partial response to erdafitinib, with a 49.6% maximum reduction in… (full text at CIViC) PMID 38603650 · Gong et al., 2024 · Open in CIViC | civic |
| AHCYL1 Fusion3 | ||||||||
| (diagnostic) | Cholangiocarcinoma | Diagnostic | B | Supports Positive | 4 | accepted | EID1847Massively parallel whole transcriptome sequencing in eight specimens from cholangiocarcinoma patients identified two fusion kinase genes, FGFR2-AHCYL1 and FGFR2-BICC1. In RT-PCR screening, the FGFR2 f… (full text at CIViC) PMID 24122810 · Arai et al., 2014 · Open in CIViC | civic |
| Erdafitinib | Intrahepatic Cholangiocarcinoma | Predictive | C | Supports Sensitivity Response | 3 | accepted | EID13164In the phase II NCI-MATCH EAY131-K2 trial, a patient with intrahepatic cholangiocarcinoma harboring an FGFR2-AHCYL1 fusion achieved a partial response to erdafitinib, with a 66.7% maximum reduction in… (full text at CIViC) PMID 38603650 · Gong et al., 2024 · Open in CIViC | civic |
| Infigratinib + PD173074Substitutes | Cholangiocarcinoma | Predictive | D | Supports | ||||
| FGFR2 Amplification + FGFR2 Fusion1 | ||||||||
| Futibatinib | Cholangiocarcinoma | Predictive | C | Supports Sensitivity Response | 1 | submitted | EID11642In a phase 1 multihistology trial of the FGFR1/2/3/4 inhibitor futibatinib, an objective response rate of 13.7% was seen with responses in a broad spectrum of tumors and across a broad spectrum of FGF… (full text at CIViC) PMID 34551969 · Meric-Bernstam et al., 2022 · Open in CIViC | civic |
| BICC1 Fusion4 | ||||||||
| (diagnostic) | Cholangiocarcinoma | Diagnostic | B | Supports Positive | 4 | accepted | EID1846Massively parallel whole transcriptome sequencing in eight specimens from cholangiocarcinoma patients identified two fusion kinase genes, FGFR2-AHCYL1 and FGFR2-BICC1. In RT-PCR screening, the FGFR2 f… (full text at CIViC) PMID 24122810 · Arai et al., 2014 · Open in CIViC | civic |
| Futibatinib | Intrahepatic Cholangiocarcinoma | Predictive | B | Supports Sensitivity Response | 3 | submitted | EID11610In a phase 2 trial (NCT02052778) of the FGFR1/2/3/4 inhibitor futibatinib in 103 patients with previously treated intrahepatic cholangiocarcinoma harboring FGFR2 fusions or rearrangements, the overal… (full text at CIViC) PMID 36652354 · Goyal et al., 2023 · Open in CIViC | civic |
| 〃 | Cholangiocarcinoma | Predictive | C | Supports Sensitivity Response | 3 | |||
| FGFR2 C383R1 | ||||||||
| Futibatinib | Cholangiocarcinoma | Predictive | C | Supports Sensitivity Response | 2 | submitted | EID11631In a phase 1 multihistology trial of the FGFR1/2/3/4 inhibitor futibatinib, an objective response rate of 13.7% was seen with responses in a broad spectrum of tumors and across a broad spectrum of FGF… (full text at CIViC) PMID 34551969 · Meric-Bernstam et al., 2022 · Open in CIViC | civic |
| CCDC6 Fusion1 | ||||||||
| Futibatinib | Cholangiocarcinoma | Predictive | C | Supports Sensitivity Response | 2 | submitted | EID11646In a phase 1 multihistology trial of the FGFR1/2/3/4 inhibitor futibatinib, an objective response rate of 13.7% was seen with responses in a broad spectrum of tumors and across a broad spectrum of FGF… (full text at CIViC) PMID 34551969 · Meric-Bernstam et al., 2022 · Open in CIViC | civic |
| DBP Fusion1 | ||||||||
| Futibatinib | Cholangiocarcinoma | Predictive | C | Supports Sensitivity Response | 2 | submitted | EID11628In a phase 1 multihistology trial of the FGFR1/2/3/4 inhibitor futibatinib, an objective response rate of 13.7% was seen with responses in a broad spectrum of tumors and across a broad spectrum of FGF… (full text at CIViC) PMID 34551969 · Meric-Bernstam et al., 2022 · Open in CIViC | civic |
| FILIP1 Fusion1 | ||||||||
| Futibatinib | Cholangiocarcinoma | Predictive | C | Supports Sensitivity Response | 1 | submitted | EID11630In a phase 1 multihistology trial of the FGFR1/2/3/4 inhibitor futibatinib, an objective response rate of 13.7% was seen with responses in a broad spectrum of tumors and across a broad spectrum of FGF… (full text at CIViC) PMID 34551969 · Meric-Bernstam et al., 2022 · Open in CIViC | civic |
| FGFR2 Fusion1 | ||||||||
| Erdafitinib | Cholangiocarcinoma | Predictive | B | Supports Sensitivity Response | 3 | submitted | EID10406Phase I dose-escalation cohort of patients with advanced solid tumors in which antineoplastic therapy (NCT01703481) was no longer effective, using pan-FGFR inhibitor erdafitinib (JNJ-42756493). Among … (full text at CIViC) PMID 31088831 · Bahleda et al., 2019 · Open in CIViC | civic |
| KIAA1217 Fusion2 | ||||||||
| Futibatinib | Intrahepatic Cholangiocarcinoma | Predictive | C | Supports Sensitivity Response | 3 | submitted | EID11611In a phase 2 trial (NCT02052778) of the FGFR1/2/3/4 inhibitor futibatinib in 103 patients with previously treated intrahepatic cholangiocarcinoma harboring FGFR2 fusions or rearrangements, the overal… (full text at CIViC) PMID 36652354 · Goyal et al., 2023 · Open in CIViC | civic |
| 〃 | Cholangiocarcinoma | Predictive | C | Supports Sensitivity Response | 1 | submitted | EID11647In a phase 1 multihistology trial of the FGFR1/2/3/4 inhibitor futibatinib, an objective response rate of 13.7% was seen with responses in a broad spectrum of tumors and across a broad spectrum of FGF… (full text at CIViC) PMID 34551969 · Meric-Bernstam et al., 2022 · Open in CIViC | civic |
| FGFR2 Mutation4 | ||||||||
| Erdafitinib | Cholangiocarcinoma | Predictive | C | Supports Sensitivity Response | 3 | submitted | EID10375Phase I dose-escalation cohort (NCT01703481) of patients with advanced solid tumors in which antineoplastic therapy was no longer effective using pan-FGFR inhibitor erdafitinib (JNJ-42756493). 1 chola… (full text at CIViC) PMID 31088831 · Bahleda et al., 2019 · Open in CIViC | civic |
| 〃 | Cholangiocarcinoma | Predictive | C | Does Not Support Sensitivity Response | 3 | submitted | EID10398Phase I dose-escalation cohort (NCT01703481) of patients with advanced solid tumors in which antineoplastic therapy was no longer effective using pan-FGFR inhibitor JNJ-42756493. 2 cholangiocarcinoma … (full text at CIViC) PMID 31088831 · Bahleda et al., 2019 · Open in CIViC | civic |
| Infigratinib | Cholangiocarcinoma | Predictive | B | Supports Sensitivity Response | 4 | |||
| CDH1 MUTATION + CDKN2A Mutation + CDKN2B Mutation + FGFR2 MutationFGFR2CDH1CDKN2ACDKN2B1 | ||||||||
| Crizotinib + Palbociclib Regimen + PazopanibCombination | Cholangiocarcinoma | Predictive | C | Supports Sensitivity Response | 2 | submitted | EID12777In a case report of metastatic cholangiocarcinoma harboring an FGFR2 W290_P307>C (16.25% Variant allele frequency) alteration together with CDH1 Q677fs*7 (23.1% VAF) and CDKN2A/B frameshift mutations … (full text at CIViC) PMID 38645422 · Aydın et al., 2024 · Open in CIViC | civic |
| NRAP Fusion1 | ||||||||
| Futibatinib | Cholangiocarcinoma | Predictive | C | Supports Sensitivity Response | 2 | submitted | EID11641In a phase 1 multihistology trial of the FGFR1/2/3/4 inhibitor futibatinib, an objective response rate of 13.7% was seen with responses in a broad spectrum of tumors and across a broad spectrum of FGF… (full text at CIViC) PMID 34551969 · Meric-Bernstam et al., 2022 · Open in CIViC | civic |
| OGA Fusion1 | ||||||||
| Ponatinib | Cholangiocarcinoma | Predictive | C | Supports Sensitivity Response | 2 | accepted | EID295In a single patient with this fusion in intrahepatic cholangiocarcinoma. Ponatinib treatment resulted in necrosis and shrinkage of the primary and metastatic sites and overall stable disease. However,… (full text at CIViC) PMID 24550739 · Borad et al., 2014 · Open in CIViC | civic |
| POC1B Fusion1 | ||||||||
| Futibatinib | Cholangiocarcinoma | Predictive | C | Supports Sensitivity Response | 1 | submitted | EID11627In a phase 1 multihistology trial of the FGFR1/2/3/4 inhibitor futibatinib, an objective response rate of 13.7% was seen with responses in a broad spectrum of tumors and across a broad spectrum of FGF… (full text at CIViC) PMID 34551969 · Meric-Bernstam et al., 2022 · Open in CIViC | civic |
| TACC3 Fusion1 | ||||||||
| Pazopanib + PonatinibSequential | Cholangiocarcinoma | Predictive | C | Supports Sensitivity Response | 3 | accepted | EID296In a 43 year old female patient with this fusion in intrahepatic cholangiocarcinoma. Both pazopanib and ponatinib separately resulted in minor tumor shrinkage. This fusion is also shown to be recurren… (full text at CIViC) PMID 24550739 · Borad et al., 2014 · Open in CIViC | civic |
| TNS1 Fusion1 | ||||||||
| Futibatinib | Cholangiocarcinoma | Predictive | C | Supports Sensitivity Response | 2 | submitted | EID11644In a phase 1 multihistology trial of the FGFR1/2/3/4 inhibitor futibatinib, an objective response rate of 13.7% was seen with responses in a broad spectrum of tumors and across a broad spectrum of FGF… (full text at CIViC) PMID 34551969 · Meric-Bernstam et al., 2022 · Open in CIViC | civic |
| TTC28 Fusion1 | ||||||||
| Futibatinib | Cholangiocarcinoma | Predictive | C | Supports Sensitivity Response | 2 | submitted | EID11645In a phase 1 multihistology trial of the FGFR1/2/3/4 inhibitor futibatinib, an objective response rate of 13.7% was seen with responses in a broad spectrum of tumors and across a broad spectrum of FGF… (full text at CIViC) PMID 34551969 · Meric-Bernstam et al., 2022 · Open in CIViC | civic |
| FGFR2 Fusion3 | ||||||||
| Derazantinib | Cholangiocarcinoma | Predictive | D | Supports Sensitivity Response | 4 | submitted | EID7969ARQ 087 exhibited inhibitory effects biochemically, in cell line and cell cycle study of FGFR2 functions. PMID 27627808 · Hall et al., 2016 · Open in CIViC | civic |
| Futibatinib | Cholangiocarcinoma | Predictive | B | Supports Sensitivity Response | 4 | submitted | EID12489The authors detail a phase 1 dose-expansion trial of a pan-FGFR inhibitor, fubatinib. Most FGFR inhibitors are reversible ATP-competitor inhibitors that tumors can acquire resistance to. Futibatinib, … (full text at CIViC) PMID 34551969 · Meric-Bernstam et al., 2022 · Open in CIViC | civic |
| 〃 | Cholangiocarcinoma | Predictive | C | Supports Sensitivity Response | 3 | submitted | EID7322 | |
| FGFR2 Fusion + FGFR2 Fusion2 | ||||||||
| Derazantinib | Intrahepatic Cholangiocarcinoma | Predictive | B | Supports Sensitivity Response | 3 | accepted | EID7329Actionable fibroblast growth factor receptor 2 (FGFR2) fusions have been identified in intrahepatic cholangiocarcinomas (iCCA). Derazantinib, a multi-kinase inhibitor with potent pan-FGFR activity sho… (full text at CIViC) PMID 30420614 · Mazzaferro et al., 2019 · Open in CIViC | civic |
| Futibatinib | Intrahepatic Cholangiocarcinoma | Predictive | A | Supports Sensitivity Response | 4 | submitted | EID11609In the phase 2 trial (NCT02052778), 103 patients (22-79 years of age) with unresectable or metastatic intrahepatic cholangiocarcinoma harboring FGFR2 fusions or rearrangements were treated with the … (full text at CIViC) PMID 36652354 · Goyal et al., 2023 · Open in CIViC | civic |
Data updated 21 hours agoSource updated unknownsource: civic (CC0)
Evidence levels, directions and ratings are those assigned by CIViC curators. "Submitted" items have not completed curation review. This is not treatment guidance.
EID5902Chemotherapy-refractory, metastatic cholangiocarcinoma with CNS involvement and a BRAF V600E mutation had a partial response at 8 weeks to dabrafenib and trametinib combination with complete radiologi… (full text at CIViC) PMID 28480077 · Kocsis et al., 2017 · Open in CIViC |
| civic |
| 〃 | Intrahepatic Cholangiocarcinoma | Predictive | C | Supports Sensitivity Response | 3 | accepted | EID5903Two cases of patients with BRAF V600E positive, refractory intrahepatic cholangiocarcinoma showed excellent clinical and radiographic response to combination dabrafenib and trametinib treatment. One p… (full text at CIViC) PMID 28078132 · Lavingia et al., 2016 · Open in CIViC | civic |
| 〃 | Intrahepatic Cholangiocarcinoma | Predictive | C | Supports Sensitivity Response | 3 | accepted | EID5904Dabrafenib and trametinib combination showed durable response for a patient with standard chemotherapy and radiation refractory, poorly differentiated, intrahepatic cholangiocarcinoma harboring BRAF V… (full text at CIViC) PMID 25435907 · Loaiza-Bonilla et al., 2014 · Open in CIViC | civic |
| Irinotecan + Panitumumab + VemurafenibCombination | Cholangiocarcinoma | Predictive | C | Supports Sensitivity Response | 3 | accepted | EID5906A 31 year old patient with metastatic cholangiocarcinoma with BRAF V600E was treated with vemurafenib, panitumumab and irinotecan triplet therapy. By 2 months, 50% reduction of tumor volume was noted,… (full text at CIViC) PMID 26687137 · Silkin et al., 2016 · Open in CIViC | civic |
| 3 |
| accepted |
EID1853Expression of the FGFR2-AHCYL1 fusion kinase in NIH3T3 cells demonstrated transforming ability that was effectively suppressed with the fibroblast growth factor receptor (FGFR) kinase inhibitors BGJ39… (full text at CIViC) PMID 24122810 · Arai et al., 2014 · Open in CIViC |
| civic |
| submitted |
EID11629In a phase 1 multihistology trial of the FGFR1/2/3/4 inhibitor futibatinib, an objective response rate of 13.7% was seen with responses in a broad spectrum of tumors and across a broad spectrum of FGF… (full text at CIViC) PMID 34551969 · Meric-Bernstam et al., 2022 · Open in CIViC |
| civic |
| Infigratinib + PD173074Substitutes | Cholangiocarcinoma | Predictive | D | Supports Sensitivity Response | 3 | accepted | EID1851Expression of the FGFR2-BICC1 fusion kinase in NIH3T3 cells demonstrated transforming ability that was effectively suppressed with the fibroblast growth factor receptor (FGFR) kinase inhibitors BGJ398… (full text at CIViC) PMID 24122810 · Arai et al., 2014 · Open in CIViC | civic |
| accepted |
EID5912Eight patients with cholangiocarcinoma haboring FGFR2 mutations received BGJ398. No response was observed, but one tumor showed a 23% reduction in size. Water fall plot of this article showed 5 patie… (full text at CIViC) PMID 29182496 · Javle et al., 2018 · Open in CIViC |
| civic |
| 〃 | Cholangiocarcinoma | Predictive | C | Supports Sensitivity Response | 2 | accepted | EID1912Phase 1 study of FGFR-inhibitor BGJ398 in advanced cancer with FGFR-aberrations. Among three patients with cholangiocarcinoma and FGFR2 aberrations (2 fusions, 1 mutation), all three had stable diseas… (full text at CIViC) PMID 27870574 · Nogova et al., 2017 · Open in CIViC | civic |
Among six patients with advanced FGFR2 fusion-positive ICC who received care at our institution after progression on BGJ398 or Debio1347 in clinical trials, four subsequently enrolled in the phase I t… (full text at CIViC)
PMID 31109923 · Goyal et al., 2019 · Open in CIViC
| civic |