Molecular Profile Detail

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Profile Name FGFR2 - BICC1
Gene Variant Detail

FGFR2 - BICC1 (gain of function)

Relevant Treatment Approaches FGFR Inhibitor (Pan) FGFR2 Inhibitor

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Molecular Profile Indication/Tumor Type Response Type Relevant Treatment Approaches Therapy Name Approval Status Evidence Type Efficacy Evidence References
FGFR2 - BICC1 intrahepatic cholangiocarcinoma sensitive FGFR Inhibitor (Pan) Infigratinib + Trametinib Preclinical Actionable In a preclinical study, Truseltiq (infigratinib) and Mekinist (trametinib) combination treatment synergistically reduced viability of cells derived from a TP53-null intrahepatic cholangiocarcinoma mouse model expressing FGFR2-BICC1 fusion and induced a partial response in 100% of tumors in a subcutaneous allograft mouse model (PMID: 33741397). 33741397
FGFR2 - BICC1 cholangiocarcinoma sensitive FGFR2 Inhibitor AZD4547 Preclinical - Cell culture Actionable In a preclinical study, cholangiocarcinoma cell lines expressing FGFR2-BICC1 were sensitive to treatment with AZD4547 in culture, demonstrating decreased cell viability, and suppression of Erk and Mapk phosphorylation (PMID: 33692336). 33692336
FGFR2 - BICC1 intrahepatic cholangiocarcinoma no benefit Trametinib Preclinical Actionable In a preclinical study, Mekinist (trametinib) treatment inhibited growth and viability of cells derived from a TP53-null intrahepatic cholangiocarcinoma mouse model expressing FGFR2-BICC1 fusion in tumoroid and 2D cell culture, but demonstrated only marginal growth inhibition in a subcutaneous allograft mouse model (PMID: 33741397). 33741397
FGFR2 - BICC1 intrahepatic cholangiocarcinoma predicted - sensitive FGFR2 Inhibitor Derazantinib Case Reports/Case Series Actionable In a Phase Ib/II trial, treatment with Derazantinib (ARQ 087) in four patients with intrahepatic cholangiocarcinoma harboring FGFR2-BICC1 resulted in one partial response, stable disease in two, and progressive disease in one (PMID: 30420614; NCT01752920). 30420614
FGFR2 - BICC1 Advanced Solid Tumor sensitive FGFR Inhibitor (Pan) Infigratinib Preclinical Actionable In a preclinical study, treatment with Truseltiq (infigratinib) prevented transformation of cells expressing FGFR2-BICC1 in culture (PMID: 24122810). 24122810
FGFR2 - BICC1 intrahepatic cholangiocarcinoma sensitive FGFR Inhibitor (Pan) Infigratinib Preclinical Actionable In a preclinical study, Truseltiq (infigratinib) treatment reduced growth and viability of cells derived from a TP53-null intrahepatic cholangiocarcinoma mouse model expressing FGFR2-BICC1 fusion in tumoroid and 2D cell culture, and resulted in a 60% partial response rate in a subcutaneous allograft mouse model (PMID: 33741397). 33741397
FGFR2 - BICC1 cholangiocarcinoma predicted - sensitive Sorafenib Case Reports/Case Series Actionable In a clinical case study, a patient with cholangiocarcinoma harboring FGFR2-BICC1 achieved a partial response with a reduction in tumor size after one month of Nexavar (sorafenib) treatment (PMID: 31807010). 31807010
FGFR2 - BICC1 intrahepatic cholangiocarcinoma predicted - sensitive FGFR Inhibitor (Pan) Futibatinib Case Reports/Case Series Actionable In a Phase II trial (FOENIX-CCA2), Futibatinib (TAS-120) demonstrated manageable toxicity profile, resulted in an objective response rate (ORR) of 37.3% (25/67), a median duration of response of 8.3 months, and a disease control rate of 82% in patients with intrahepatic cholangiocarcinoma harboring FGFR2 fusions (82%) or other rearrangements (18%), ORR was 33.3% (5/15) in patients harboring FGFR2-BICC1 fusion (Annals of Oncology (2020) 31 (suppl_4): S261-S262; NCT02052778). detail...
FGFR2 - BICC1 intrahepatic cholangiocarcinoma predicted - sensitive FGFR Inhibitor (Pan) Futibatinib Case Reports/Case Series Actionable In a Phase I trial (FOENIX-101), Futibatinib (TAS-120) treatment resulted in a partial response in two patients with intrahepatic cholangiocarcinoma harboring FGFR2-BICC1 fusion (PMID: 32622884; NCT02052778). 32622884
FGFR2 - BICC1 cholangiocarcinoma sensitive FGFR Inhibitor (Pan) Infigratinib Preclinical - Cell culture Actionable In a preclinical study, cholangiocarcinoma cell lines expressing FGFR2-BICC1 were sensitive to treatment with Truseltiq (infigratinib) in culture, demonstrating decreased cell viability, and suppression of Erk and Mapk phosphorylation (PMID: 33692336). 33692336
FGFR2 - BICC1 Advanced Solid Tumor sensitive FGFR Inhibitor (Pan) Erdafitinib Preclinical - Cell culture Actionable In a preclinical study, Balversa (erdafitinib) inhibited Fgfr phosphorylation and downstream signaling, resulted in growth inhibition of transformed cells expressing FGFR2-BICC1 in culture (PMID: 28416604). 28416604
FGFR2 - BICC1 Advanced Solid Tumor sensitive PD173074 Preclinical Actionable In a preclinical study, treatment with PD173074 prevented transformation of cells expressing FGFR2-BICC1 in culture (PMID: 24122810). 24122810
FGFR2 - BICC1 cholangiocarcinoma sensitive FGFR2 Inhibitor Pemigatinib Phase II Actionable In a Phase II (FIGHT-202) trial, Pemazyre (pemigatinib) treatment resulted in an objective response rate (ORR) of 35.5% (38/107, 3 complete response, 35 partial response) in patients with advanced cholangiocarcinoma harboring FGFR2 fusions or rearrangements, 29% (31/107) of the patients harbored FGFR2-BICC1 and achieved an ORR (32.3%, 10/31) and a median progression-free survival (6.80 vs 6.93 months) comparable to the entire group (PMID: 32203698; NCT02924376). 32203698
Clinical Trial Phase Therapies Title Recruitment Status Covered Countries Other Countries