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 |
|---|---|---|---|---|---|---|---|---|
| NRG1 Fusion + KRAS WildtypeNRG1KRAS1 | ||||||||
| Afatinib | Pancreatic Ductal Adenocarcinoma | Predictive | C | Supports Sensitivity Response | 3 | accepted | EID7493Whole genome sequencing and transcriptomic analysis were performed on 47 advanced PDAC patients. 3/47 patients were KRAS wildtype and all three of these cases harbored a NRG1 fusion. In a 54-year-old … (full text at CIViC) PMID 31068372 · Jones et al., 2019 · Open in CIViC | civic |
| ATM R2034Ter1 | ||||||||
| Olaparib | Pancreatic Ductal Adenocarcinoma | Predictive | C | Supports Sensitivity Response | 1 | submitted | EID13230A 77 year old man with metastatic pancreatic ductal adenocarcinoma with somatic ATM R2034Ter mutation received off label olaparib after prior chemotherap. His disease remained stable for 18 months. PMID 40273902 · Vornhülz et al., 2025 · Open in CIViC | civic |
| 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 |
| NRG1 Fusion + KRAS WildtypeNRG1KRAS1 | ||||||||
| Afatinib | Pancreatic Ductal Adenocarcinoma | Predictive | C | Supports Sensitivity Response | 3 | accepted | EID7492Whole genome sequencing and transcriptomic analysis were performed on 47 advanced PDAC patients. 3/47 patients were KRAS wildtype and all three of these cases harbored a NRG1 fusion. A 59-year-old met… (full text at CIViC) PMID 31068372 · Jones et al., 2019 · 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 | 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 | 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 | |||
| BRCA1 Loss-of-function1 | ||||||||
| Olaparib | Pancreatic Adenocarcinoma | Predictive | A | Supports Sensitivity Response | 5 | accepted | EID7384In the phase 3 POLO trial patients who had germline BRCA1/2 mutation and metastatic pancreatic adenocarcinoma that had not progressed during first-line platinum-based chemotherapy were allocated to ma… (full text at CIViC) PMID 31157963 · Golan et al., 2019 · Open in CIViC | civic |
| BRCA1 Mutation + BRCA2 Mutation + PALB2 MutationBRCA2BRCA1PALB21 | ||||||||
| Rucaparib | Pancreatic Adenocarcinoma | Predictive | B | Supports Sensitivity Response | 3 | submitted | EID11317In this phase II study, 46 patients were enrolled (42 were evaluable; 27 gBRCA2, seven gBRCA1, six gPALB2, and two sBRCA2) and their chemotherapy was discontinued; patients then received rucaparib 600… (full text at CIViC) PMID 33970687 · Reiss et al., 2021 · Open in CIViC | civic |
| BRCA2 Loss-of-function1 | ||||||||
| Olaparib | Pancreatic Adenocarcinoma | Predictive | A | Supports Sensitivity Response | 5 | accepted | EID7385In the phase 3 POLO trial patients who had germline BRCA1/2 mutation and metastatic pancreatic adenocarcinoma that had not progressed during first-line platinum-based chemotherapy were allocated to ma… (full text at CIViC) PMID 31157963 · Golan et al., 2019 · Open in CIViC | civic |
| CD44 CD44v6+/CD44s-1 | ||||||||
| (prognostic) | Pancreatic Carcinoma | Prognostic | B | Supports Poor Outcome | 4 | submitted | EID9433In a multivariate analysis from a clinical trial of 101 patients, CD44v6 expression combined with CD44s non-expression is significantly correlated with a reduced survival (p= 0.003). PMID 24708709 · Li et al., 2014 · 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 |
| BEND2 Fusion1 | ||||||||
| (oncogenic) | Pancreatic Neuroendocrine CarcinomaALIAS | Oncogenic | C | Supports Oncogenicity | 1 | accepted | EID9219A 35 year-old patient presented with a grade 3 pancreatic neuroendocrine tumor and liver metastasis. Whole genome sequencing and RNAseq revealed a previously undescribed somatic translocation between … (full text at CIViC) PMID 31160355 · Williamson et al., 2019 · Open in CIViC | civic |
| CIP2A EXPRESSION1 | ||||||||
| (prognostic) | Pancreatic Ductal Adenocarcinoma | Prognostic | B | Supports Poor Outcome | 3 | accepted | EID1117Expression of the protein CIP2A was investigated in 72 cases of pancreatic ductal adenocarcinoma and 27 cases of adjacent tissues. Immunohistochemistry revealed a significant difference in expression … (full text at CIViC) PMID 26894380 · Xu et al., 2016 · Open in CIViC | civic |
| CIP2A Underexpression1 | ||||||||
| Gemcitabine | Pancreatic Ductal Adenocarcinoma | Predictive | D | Supports Sensitivity Response | 3 | accepted | EID1118Researchers studied the effect of CIP2A expression in relation to gemcitabine sensitivity via SW1990 cell lines. SW1990 cells with CIP2A expression knocked-down displayed lower IC50 (p=.024), indicat… (full text at CIViC) PMID 26894380 · Xu et al., 2016 · Open in CIViC | civic |
| EGFR L747_P753delinsS1 | ||||||||
| Erlotinib | Pancreatic Adenocarcinoma | Predictive | C | Supports Sensitivity Response | 3 | accepted | EID6005Patients with recurrent pancreatic adenocarcinoma was treated with gemcitabine + nab-paclitaxel, FOLFIRINOX and docetaxel + irinotecan. After failure of these treatment, a tumor specimen from the panc… (full text at CIViC) PMID 28874593 · Cecchini et al., 2017 · Open in 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 T790M2 | ||||||||
| (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 |
| Osimertinib | Pancreatic Adenocarcinoma | Predictive | C | Does Not Support Sensitivity Response | 2 | accepted | EID6006Patients with recurrent pancreatic adenocarcinoma was treated with gemcitabine + nab-paclitaxel, FOLFIRINOX and docetaxel + irinotecan. After failure of these treatment, a tumor specimen from the panc… (full text at CIViC) PMID 28874593 · Cecchini et al., 2017 · Open in CIViC | civic |
| NTRK3 Fusion1 | ||||||||
| (oncogenic) | Pancreatic Ductal Adenocarcinoma | Oncogenic | C | Supports Oncogenicity | 1 | accepted | EID10484Whole genome sequencing and RNAseq were performed on 400 patients with resected or locally advanced/metastatic Pancreatic Ductal Adenocarcinoma (PDAC). Immunohistochemistry (IHC) analysis was performe… (full text at CIViC) PMID 34583947 · Allen et al., 2023 · Open in CIViC | civic |
| ERBB2 Amplification3 | ||||||||
| (oncogenic) | Pancreatic Ductal Adenocarcinoma | Oncogenic | D | Supports Oncogenicity | 3 | submitted | EID11563The ERRB2 amplification effect in PDAC was studied through CAPAN1 cells and ERBB2-KO (knockout) CAPAN1 cells that were KO using CRISPR (Figure 4C, Supp Table 4), with a 90% reduction of ERBB2 compared… (full text at CIViC) PMID 31046123 · Li et al., 2020 · Open in CIViC | civic |
| Afatinib | Pancreatic Adenocarcinoma | Predictive | D | Supports Sensitivity Response | 2 | accepted | EID962Afatinib was growth-inhibitory for three pancreatic cancer cell lines but cytotoxic only toward BxPC3 (KRAS wt) and Capan-2 (KRAS mut) cells, both of which express high levels of EGFR, ERBB2, and ERBB… (full text at CIViC) PMID 26668065 · Huguet et al., 2016 · Open in CIViC | civic |
| Pertuzumab + TrastuzumabCombination | Cholangiocarcinoma | Predictive | C | Supports | ||||
| ERBB2 E717D1 | ||||||||
| (oncogenic) | Pancreatic Ductal Adenocarcinoma | Oncogenic | D | Supports Oncogenicity | 3 | submitted | EID11555HPNE cells infected with lentiviral vectors containing the variant did show significant phosphorylation of ERBB2 (Supplementary Figure 1) compared to WT (Figure 2A and B). The variant also exhibits an… (full text at CIViC) PMID 31046123 · Li et al., 2020 · 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 |
| ERBB2 L755S1 | ||||||||
| (oncogenic) | Pancreatic Ductal Adenocarcinoma | Oncogenic | D | Supports Oncogenicity | 3 | submitted | EID11557HPNE cells infected with lentiviral vectors containing the variant did show significant phosphorylation of ERBB2 (Supplementary Figure 1) compared to WT (Figure 2A and B). The variant also exhibits an… (full text at CIViC) PMID 31046123 · Li et al., 2020 · Open in CIViC | civic |
| ERBB2 L755S + KRAS G12DERBB2KRAS1 | ||||||||
| (oncogenic) | Pancreatic Ductal Adenocarcinoma | Oncogenic | D | Supports Oncogenicity | 3 | submitted | EID11558KRAS G12D-transformed HPNE cells with lentiviral vectors containing the variant significantly increased colony formation in number and size in soft agar compared to infected HPNE cells with the varian… (full text at CIViC) PMID 31046123 · Li et al., 2020 · Open in CIViC | civic |
| ERBB2 Q679L1 | ||||||||
| (oncogenic) | Pancreatic Ductal Adenocarcinoma | Oncogenic | D | Supports Oncogenicity | 3 | submitted | EID11553HPNE cells infected with lentiviral vectors containing the variant did show significant phosphorylation of ERBB2 (Supplementary Figure 1) compared to WT (Figure 2A and B). The variant also exhibits an… (full text at CIViC) PMID 31046123 · Li et al., 2020 · Open in CIViC | civic |
| ERBB2 R678Q1 | ||||||||
| (oncogenic) | Pancreatic Ductal Adenocarcinoma | Oncogenic | D | Supports Oncogenicity | 3 | submitted | EID11551HPNE cells infected with lentiviral vectors containing the variant did show significant phosphorylation of ERBB2 (Supplementary Figure 1) compared to WT (Figure 2A and B). The variant also exhibits an… (full text at CIViC) PMID 31046123 · Li et al., 2020 · Open in CIViC | civic |
| ERBB2 R678Q + KRAS G12DERBB2KRAS1 | ||||||||
| (oncogenic) | Pancreatic Ductal Adenocarcinoma | Oncogenic | D | Supports Oncogenicity | 3 | submitted | EID11552KRAS G12D-transformed HPNE cells with lentiviral vectors containing the variant significantly increased colony formation in number and size in soft agar compared to infected HPNE cells with the varian… (full text at CIViC) PMID 31046123 · Li et al., 2020 · Open in CIViC | civic |
| ERBB2 S310F1 | ||||||||
| (oncogenic) | Pancreatic Ductal Adenocarcinoma | Oncogenic | D | Supports Oncogenicity | 3 | submitted | EID11547HPNE cells infected with lentiviral vectors containing the variant did show significant phosphorylation of ERBB2 (Supplementary Figure 1) compared to WT (Figure 2A and B). The variant also exhibits an… (full text at CIViC) PMID 31046123 · Li et al., 2020 · Open in CIViC | civic |
| ERBB2 S423R1 | ||||||||
| (oncogenic) | Pancreatic Ductal Adenocarcinoma | Oncogenic | D | Supports Oncogenicity | 3 | submitted | EID11549HPNE cells infected with lentiviral vectors containing the variant did show significant phosphorylation of ERBB2 (Supplementary Figure 1) compared to WT (Figure 2A and B). The variant also exhibits an… (full text at CIViC) PMID 31046123 · Li et al., 2020 · Open in CIViC | civic |
| ERBB2 V777L2 | ||||||||
| (oncogenic) | Pancreatic Ductal Adenocarcinoma | Oncogenic | D | Supports Oncogenicity | 3 | submitted | EID11559HPNE cells infected with lentiviral vectors containing the variant did show significant phosphorylation of ERBB2 (Supplementary Figure 1) compared to WT (Figure 2A and B). The variant also exhibits an… (full text at CIViC) PMID 31046123 · Li et al., 2020 · Open in CIViC | civic |
| Neratinib | Pancreatic Ductal Adenocarcinoma | Predictive | D | Supports Sensitivity Response | 3 | submitted | EID11569Neratinib suppresses anchorage-independent growth in transduced HPNE cells with the variant in a concentration-dependent manner (Figure 6B–C) with complete inhibition at a concentration of 0.5 µM (Fig… (full text at CIViC) PMID 31046123 · Li et al., 2020 · Open in CIViC | civic |
| ERBB2 V777L + KRAS G12CERBB2KRAS1 | ||||||||
| ARS-1620 + NeratinibCombination | Pancreatic Ductal Adenocarcinoma | Predictive | D | Supports Sensitivity Response | 3 | submitted | EID11572When tested in MIAPaCa2 (PDAC cell line and KRAS G12C) cells, Neratinib and ARS-1620 strongly inhibit AKT and MAPK phosphorylation (Figure 6F). Also, MIAPaCa2 cell line with the variant in exposure to… (full text at CIViC) PMID 31046123 · Li et al., 2020 · Open in CIViC | civic |
| ERBB2 V777L + KRAS G12DERBB2KRAS1 | ||||||||
| (oncogenic) | Pancreatic Ductal Adenocarcinoma | Oncogenic | D | Supports Oncogenicity | 3 | submitted | EID11560KRAS G12D-transformed HPNE cells with lentiviral vectors containing the variant significantly increased colony formation in number and size in soft agar compared to infected HPNE cells with the varian… (full text at CIViC) PMID 31046123 · Li et al., 2020 · Open in CIViC | civic |
| ERBB2 V842I1 | ||||||||
| (oncogenic) | Pancreatic Ductal Adenocarcinoma | Oncogenic | D | Supports Oncogenicity | 3 | submitted | EID11561HPNE cells infected with lentiviral vectors containing the variant did show significant phosphorylation of ERBB2 (Supplementary Figure 1) compared to WT (Figure 2A and B). The variant also exhibits an… (full text at CIViC) PMID 31046123 · Li et al., 2020 · Open in CIViC | civic |
| ERBB2 V842I1 | ||||||||
| Neratinib | Pancreatic Ductal Adenocarcinoma | Predictive | D | Supports Sensitivity Response | 3 | submitted | EID11570Neratinib suppresses anchorage-independent growth in transduced HPNE cells with the variant in a concentration-dependent manner (Figure 6B–C) with complete inhibition at a concentration of 0.5 µM (Fig… (full text at CIViC) PMID 31046123 · Li et al., 2020 · Open in CIViC | civic |
| ERBB2 V842I + KRAS G12DERBB2KRAS1 | ||||||||
| (oncogenic) | Pancreatic Ductal Adenocarcinoma | Oncogenic | D | Supports Oncogenicity | 3 | submitted | EID11562KRAS G12D-transformed HPNE cells with lentiviral vectors containing the variant significantly increased colony formation in number and size in soft agar compared to infected HPNE cells with the varian… (full text at CIViC) PMID 31046123 · Li et al., 2020 · Open in CIViC | civic |
| ERBB3 EXPRESSION1 | ||||||||
| 9F7-F11 + PertuzumabCombination | Pancreatic Ductal Adenocarcinoma | Predictive | D | Supports Sensitivity Response | 3 | accepted | EID865In mice xenografts expressing ERBB3 (HER3) the combination of ERBB2 (HER2) and ERBB3 inhibitors pertuzumab and 9F7-F11enhanced tumor inhibition and the median survival time. Sensitivity to single-agen… (full text at CIViC) PMID 25216528 · Thomas 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 | ||||
Data updated 13 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.
| 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 |
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| 〃 | 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 |
| Dabrafenib/Trametinib Regimen | Pancreatic Adenocarcinoma | Predictive | C | Supports Sensitivity Response | 2 | submitted | EID12811A 78-year-old Chinese female patient presented with BRAF V600E-mutant metastatic pancreatic adenocarcinoma. The patient received two cycles of chemotherapy. Following the second incomplete cycle of ch… (full text at CIViC) PMID 41367868 · Liu et al., 2025 · 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 |
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 |
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| 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 |
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