Subtype
Pancreatobiliary Carcinoma
CI-CAN-00001365Explore in graph →
- NCIt
- C166418
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Subtype
CI-CAN-00001365Explore in graph →
Variants & evidence
129 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 |
|---|---|---|---|---|---|---|---|---|
| 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 Fusion8 | ||||||||
| 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 |
| 〃 | Intrahepatic Cholangiocarcinoma | Predictive | B | Supports | ||||
| FGFR2 W290C1 | ||||||||
| Futibatinib | Cholangiocarcinoma | Predictive | C | Supports Sensitivity Response | 1 | submitted | EID11633In 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 |
| WAC Fusion1 | ||||||||
| Futibatinib | Cholangiocarcinoma | Predictive | C | Supports Sensitivity Response | 1 | submitted | EID11643In 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 Y376C1 | ||||||||
| AZD-4547 | Intrahepatic Cholangiocarcinoma | Predictive | C | Does Not Support Sensitivity Response | 1 | submitted | EID8317The phase II NCI-MATCH trial (EAY131) subprotocol W assessed the FGFR1-3 selective TKI AZD-4547 in 48 patients with FGFR1/2 amplifications, FGFR2/3 SNV or FGFR1/3 fusions. The partial response overall… (full text at CIViC) PMID 32463741 · Chae et al., 2020 · Open in CIViC | civic |
| FGFR3 K508M1 | ||||||||
| (oncogenic) | Pancreatic Ductal Adenocarcinoma | Oncogenic | D | Does Not Support Oncogenicity | 2 | submitted | EID11798The study analysed the expression of the two splice variants of FGFR3 (FGFR3-IIIb and FGFR3-IIIc/FGFR3-IIIc-KD) in two pancreatic ductal adenocarcinoma cell lines (Capan-2 and BxPC-3). The FGFR3-IIIc-… (full text at CIViC) PMID 23902722 · Lafitte et al., 2013 · Open in CIViC | civic |
| GNAS c.393T>C1 | ||||||||
| (prognostic) | Intrahepatic Cholangiocarcinoma | Prognostic | B | Supports Poor Outcome | 3 | accepted | EID1996Intrahepatic cholangiocarcinomas of patients with homozygous TT genotypes revealed a higher proliferation rate and a lower apoptotic rate.This study demonstrated a significant association of both dise… (full text at CIViC) PMID 17356712 · Schmitz et al., 2007 · Open in CIViC | civic |
| NTRK1 Fusion1 | ||||||||
| Entrectinib + LarotrectinibSequential | Pancreatic Ductal Adenocarcinoma | Predictive | C | Supports Sensitivity Response | 2 | submitted | EID13049This case study reports a 78 year old patient with pancreatic adenocarcinoma harboring a GP2::NTRK1 gene fusion characterized by an in-frame rearrangement involving exon 2 of GP2 and exon 8 of NTRK1. … (full text at CIViC) PMID 42412359 · Tazuma et al., 2026 · Open in CIViC | civic |
| HIF1A 3' UTR Polymorphism1 | ||||||||
| (prognostic) | Pancreatic Adenocarcinoma | Prognostic | B | Supports Poor Outcome | 2 | accepted | EID1031The HIF1A rs2057482 SNP was genotyped in 410 pancreatic ductal adenocarcinoma cases and 490 healthy controls. The CC genotype was significantly correlated with PDAC risk (OR = 1.719, 95% CI: 1.293-2.2… (full text at CIViC) PMID 26872370 · Wang et al., 2016 · Open in CIViC | civic |
| HSPB1 EXPRESSION1 | ||||||||
| Gemcitabine | Pancreatic Ductal Adenocarcinoma | Predictive | D | Supports Sensitivity Response | 2 | accepted | EID939HSP27 (HSPB1) expression in pancreatic adenocarcinoma cell lines was associated with sensitivity to treatment with gemcitabine. PMID 22004109 · Schäfer et al., 2012 · Open in CIViC | civic |
| IDH1 Mutation1 | ||||||||
| Ivosidenib | Cholangiocarcinoma | Predictive | B | Supports Sensitivity Response | 3 | accepted | EID7447In a phase1 trial, 73 patients with cholangiocarcinoma harboring IDH1 mutations were treated with mutant IDH1 inhibitor ivosidenib. The response rate was 5%, median progression free survival was 3.8 m… (full text at CIViC) PMID 31300360 · Lowery et al., 2019 · Open in CIViC | civic |
| IDH1 R1322 | ||||||||
| Dasatinib | Intrahepatic Cholangiocarcinoma | Predictive | D | Supports Sensitivity Response | 4 | accepted | EID6439Through a high-throughput drug screen of a large panel of cancer cell lines, including 17 biliary tract cancers, this work found that IDH mutant (IDHm) ICC cells demonstrate a striking response to the… (full text at CIViC) PMID 27231123 · Saha et al., 2016 · Open in CIViC | civic |
| Ivosidenib | Cholangiocarcinoma | Predictive | A | Supports Sensitivity Response | 5 | accepted | EID10886The multicenter randomized double-blind phase 3 study included patients with IDH1-mutant cholangiocarcinoma who had progressed on previous therapy. Patients were randomly assigned to Ivosidenib or pla… (full text at CIViC) PMID 32416072 · Abou-Alfa et al., 2020 · Open in CIViC | civic |
| IDH1 R132H1 | ||||||||
| Ivosidenib | Pancreatic Ductal Adenocarcinoma | Predictive | C | Does Not Support Sensitivity Response | 2 | accepted | EID5987A 48-year-old female was diagnosed with metastatic pancreatic ductal adenocarcinoma and subsequently started on standard of care chemotherapy, during which her hepatic lesions progressed. Detailed mol… (full text at CIViC) PMID 27466707 · Brody et al., 2018 · Open in CIViC | civic |
| IGF2 Overexpression1 | ||||||||
| Ganitumab + GemcitabineCombination | Pancreatic Adenocarcinoma | Predictive | B | Supports Sensitivity Response | 4 | submitted | EID7000In pancreatic cancer patients, ganitumab was associated with improved OS compared with placebo. The effect of ganitumab on improved OS was stronger for patients with higher levels of IGF-2. As Figur… (full text at CIViC) PMID 23741071 · McCaffery et al., 2013 · Open in CIViC | civic |
| ALK Fusion1 | ||||||||
| Alectinib | Pancreatic Acinar Cell CarcinomaALIAS | Predictive | C | Supports Sensitivity Response | 3 | accepted | EID10327Case report of a 62-year old male patient diagnosed with pancreatic acinar cell carcinoma. Liver metastases were diagnosed and treatment was started with gemcitabine plus nab-paclitaxel regimen. After… (full text at CIViC) PMID 35005993 · Gaule et al., 2022 · Open in CIViC | civic |
| KRAS Exon 2 Mutation2 | ||||||||
| (prognostic) | Pancreatic Carcinoma | Prognostic | B | Supports Poor Outcome | 3 | accepted | EID917KRAS exon 2 was mutated in 121 of 173 patients with advanced pancreatic cancer. Patients with KRAS wildtype had an improved OS (HR 1.68, p = 0.005). PMID 23435671 · Boeck et al., 2013 · Open in CIViC | civic |
| Erlotinib | Pancreatic Carcinoma | Predictive | B | Does Not Support Resistance | 3 | accepted | EID916KRAS exon 2 was mutated in 121 of 173 (70%) patients with advanced pancreatic cancer and did not show an association with response to erlotinib treatment. PMID 23435671 · Boeck et al., 2013 · Open in CIViC | civic |
Data updated 22 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.
| 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 |
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| 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 |
| 3 |
| submitted |
EID11612In 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 | submitted | EID11632In 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 | Cholangiocarcinoma | Predictive | A | Supports Sensitivity Response | 4 | accepted | EID11230This open-label phase 2 trial included 122 patients with histologically or cytologically confirmed, locally advanced or metastatic cholangiocarcinoma, 108 of whom had an FGFR2 fusion or rearrangement.… (full text at CIViC) PMID 34358484 · Javle et al., 2021 · Open in CIViC | civic |
| 〃 | Cholangiocarcinoma | Predictive | B | Supports Sensitivity Response | 4 | accepted | EID5908This Phase II trial of BGJ398 for FGFR2 aberant cholangiocarcinoma included 48 patients with FGFR2 fusions. Among these patients with FGFR2 fusions, eight showed tumor response (18.8%) and thirty-six … (full text at CIViC) PMID 29182496 · Javle et al., 2018 · Open in CIViC | civic |
| 〃 | Cholangiocarcinoma | Predictive | C | Supports Sensitivity Response | 2 | accepted | EID1913Phase 1 study of FGFR-inhibitor BGJ398 (infigratinib) in advanced cancer with FGFR-aberrations (n=132). Two cholangiocarcinoma patients harbored FGFR2 fusions. Both were treated at BGJ398 doses >100mg… (full text at CIViC) PMID 27870574 · Nogova et al., 2017 · Open in CIViC | civic |
| Pemigatinib | Cholangiolocellular Small Duct Intrahepatic CholangiocarcinomaALIAS | Predictive | A | Supports Sensitivity Response | 4 | accepted | EID8173In a multicentre, open-label, single-arm, phase 2 study (FIGHT-202), patients with disease progression following at least one previous treatment received a starting dose of 13·5 mg oral pemigatinib on… (full text at CIViC) PMID 32203698 · Abou-Alfa et al., 2020 · Open in CIViC | civic |