Gene

FGFR3

fibroblast growth factor receptor 3

Explore in graph →CI-GENE-00001926HGNC:3690 ENSG00000068078 NCBI 2261 CIViC Cancer geneApproved

Curated evidence

Clinical evidence (214)

CIViC items involving this gene, grouped by molecular profile and therapy, with native levels and directions. 50 items per page.

civicCuratedShowing 1–50 of 214 evidence items · levels, directions and significance as curated at the source; each row links to its CIViC record.

Therapy	Cancer	Type	Level	Direction · significance	Rating (1–5)	Status	Evidence	Source
FGFR3 Fusion1
Midostaurin	Myeloproliferative NeoplasmCURATED_BROADER	Predictive	D	Supports Sensitivity Response	3

Cohorts

Alteration frequency by cohort (100)

Frequency = cases affected / cases profiled within one cohort. Cohorts are never pooled.

cbioportalObserved100 cohort rows (showing 50, sorted by frequency) · frequency = affected / profiled, as published by each cohort; cohorts are not pooled. Hover a row badge for its release.

Cohort	Mapped cancer	Alteration	Affected (n)	Profiled (n)	Frequency (%)	Rank in cohort	Source
Non-Small Cell Lung Cancer (MSK, 2026) alk_msk_2026	Lung Non-Small Cell Carcinoma	Ssm	2	90	2.2%

Literature

Linked publications (36)

25 per page, newest first.

pubmedPublishedShowing 1–25 of 36 publications · bibliographic data as recorded by PubMed; abstracts are not reproduced.

Erdafitinib in Locally Advanced or Metastatic Urothelial Carcinoma.
· N Engl J Med · 2019 · PMID 31340094 · validated civic_curation pubmed
FGFR3 mutational status and protein expression in patients with bladder cancer in a Jordanian population.
· Cancer Epidemiol · 2010 · PMID 20542753 · validated civic_curation pubmed
Evaluation of BGJ398, a Fibroblast Growth Factor Receptor 1-3 Kinase Inhibitor, in Patients With Advanced Solid Tumors Harboring Genetic Alterations in Fibroblast Growth Factor Receptors: Results of a Global Phase I, Dose-Escalation and Dose-Expansion Study.
· J Clin Oncol · 2017 · PMID 27870574 · validated civic_curation pubmed
Somatic FGFR3 Mutations Distinguish a Subgroup of Muscle-Invasive Bladder Cancers with Response to Neoadjuvant Chemotherapy.
· EBioMedicine · 2018 · PMID 29941343

FGFR1 Amplification + FGFR2 Amplification + FGFR3 Amplification + FGFR4 AmplificationFGFR1 FGFR2 FGFR3 FGFR41
Erdafitinib	Malignant NeoplasmALIAS	Predictive	B	Does Not Support Sensitivity Response	3	submitted	EID12024 In this phase II trial, 35 patients harbouring tumours with FGFR1-4 amplifications were treated with FGFR1-4 inhibitor, erdafitinib. 33 of the 35 patients were eligible and treated, while biopsies wer… (full text at CIViC) PMID 38603651 · Gong et al., 2024 · Open in CIViC	civic

FGFR1 Amplification + FGFR2 Amplification + FGFR3 Amplification + FGFR4 AmplificationFGFR1 FGFR2 FGFR3 FGFR41

Erdafitinib

Malignant NeoplasmALIAS

Predictive

Does Not Support Sensitivity Response

submitted

EID12024

In this phase II trial, 35 patients harbouring tumours with FGFR1-4 amplifications were treated with FGFR1-4 inhibitor, erdafitinib. 33 of the 35 patients were eligible and treated, while biopsies wer… (full text at CIViC)

PMID 38603651 · Gong et al., 2024 · Open in CIViC

FGFR3 A391E7
(functional)	—UNRESOLVED	Functional	D	Supports Gain Of Function	4	accepted	EID10513 FRET was used to compare the structural changes in FGFR dimers when FGF1 and FGF2 bind, and found that FGF2 bound dimers were much closer to each other than FGF1. The authors also looked at FGFR3 phos… (full text at CIViC) PMID 26725515 · Sarabipour et al., 2016 · Open in CIViC	civic
〃	—UNRESOLVED	Functional	D	Supports Gain Of Function	3	submitted	EID10395 While receptor tyrosine kinases (RTKs) like FGFR3 form both homodimers and heterodimers (often in the same family), heterodimerization of RTKs is considered to be a method of greater receptor activati… (full text at CIViC) PMID 27927983 · Del Piccolo et al., 2017 · Open in CIViC	civic
〃	—UNRESOLVED	Functional	D	Supports Gain Of Function	3	submitted	EID10509 Common bladder cancer and Crouzon syndrome with acanthosis nigricans variant FGFR3 A391E dimerization was tested in plasma membrane derived vesicles, and quantified via QI-FRET and Intrinsic FRET. Th… (full text at CIViC) PMID 26244699 · Sarabipour et al., 2015 · Open in CIViC	civic
〃	—UNRESOLVED	Functional	D	Supports Gain Of Function	3	submitted	EID10654 Crouzon syndrome with acanthosis nigricans mutation FGFR3 A391E activation was tested in the presence of ligand fgf1 via Western blots as well as testing dimerization, phosphorylation and ligand bindi… (full text at CIViC) PMID 23437153 · Chen et al., 2013 · Open in CIViC	civic
(oncogenic)	Malignant NeoplasmALIAS	Oncogenic	D	Supports Oncogenicity	3	submitted	EID12849 The transmembrane domain mutation A391E was tested in a series of assays for an evaluation of oncogenicity. A391E was stably transfected into 3T3 cells along with WT controls. TAS (Transformation Ac… (full text at CIViC) PMID 34272467 · Nakamura et al., 2021 · Open in CIViC	civic
AZD-4547	Transitional Cell Carcinoma	Predictive	C	Supports Sensitivity Response	1	submitted	EID8315 The 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
Erdafitinib	Malignant NeoplasmALIAS	Predictive	D	Supports Sensitivity Response	3	submitted	EID13208 NIH3T3 cells expressing FGFR3 A391E were sensitive to erdafitinib in an in vitro cell proliferation assay. IC50 calculated by comparison to a vehicle control was 5.1nM, which was lower than IC50 recor… (full text at CIViC) PMID 34272467 · Nakamura et al., 2021 · Open in CIViC	civic

FGFR3 Amplification3
Pazopanib	Malignant Breast Neoplasm	Predictive	C	Does Not Support Sensitivity Response	3	accepted	EID7955 Patients with breast cancer had their tumor samples analyzed by Foundation Medicine in order to determine an appropriate clinical trial with targeted therapy or retrospectively to correlate with respo… (full text at CIViC) PMID 27489863 · Wheler et al., 2016 · Open in CIViC	civic
〃	Ewing Sarcoma	Predictive	C	Supports Sensitivity Response	2	submitted	EID7835 Clinical trial of soft tissue sarcoma patients found no responses to pazopanib + trametinib combination in Ewing sarcoma patients. Comparison was done to previously treated Ewing sarcoma that responde… (full text at CIViC) PMID 28377484 · Subbiah et al., 2017 · Open in CIViC	civic
Ponatinib	Malignant Gastric Neoplasm	Predictive	D	Supports Sensitivity Response	3	rejected	EID8061 In a panel of 14 cell lines representing multiple tumor types (endometrial, bladder, gastric, breast, lung, and colon) and containing FGFRs dysregulated by a variety of mechanisms, ponatinib inhibited… (full text at CIViC) PMID 22238366 · Gozgit et al., 2012 · Open in CIViC	civic

FGFR3 F384L3
(predisposing)	HypochondroplasiaUNRESOLVED	Predisposing	C	Supports Predisposition	1	submitted	EID9707 A rare variant was found through Sanger sequencing on affected members of a family with short stature. The segregation of the variant was not entirely related to the phenotype since it was present in … (full text at CIViC) PMID 25777271 · Kant et al., 2015 · Open in CIViC	civic
Infigratinib	Transitional Cell Carcinoma	Predictive	C	Does Not Support Sensitivity Response	2	submitted	EID6415 In this one-arm study, 67 patients with metastastic urothelial carcinoma and diverse FGFR3 alterations were treated with pan-FGFR Inhibitor BGJ398. The majority of patients (70.1%) had received two or… (full text at CIViC) PMID 29848605 · Pal et al., 2018 · Open in CIViC	civic
Midostaurin	Multiple Myeloma	Predictive	D	Supports Sensitivity Response	2	submitted	EID10410 Cell line LP1 expressing FGFR3 F384L was treated with small molecule inhibitor PKC412, with relative viability rising above 1.1/1.0 at both 50 nM and 100 nM. Relative viability dropped to 0.96/1.0 at … (full text at CIViC) PMID 16091734 · Chen et al., 2005 · Open in CIViC	civic

FGFR3 G370C14
(oncogenic)	Bladder Urothelial Carcinoma	Oncogenic	C	Supports Oncogenicity	3	submitted	EID13142 In a pan-cancer analysis of 10,967 TCGA tumor samples across 32 cancer types, FGFR3 G370C was identified in 5 bladder urothelial carcinoma samples and was classified as an oncogenic FGFR3 mutation. G3… (full text at CIViC) PMID 34160364 · Li et al., 2021 · Open in CIViC	civic
〃	Malignant NeoplasmALIAS	Oncogenic	C	Supports Oncogenicity	3	submitted	EID13143 In a targeted next-generation sequencing analysis of 4,853 solid tumors, FGFR3 G370C was identified as a somatic mutation in 3 cases: one cutaneous squamous cell carcinoma, one non-small cell lung car… (full text at CIViC) PMID 26373574 · Helsten et al., 2016 · Open in CIViC	civic
〃	Bladder Urothelial Carcinoma	Oncogenic	C	Supports Oncogenicity	3	submitted	EID13144 In an analysis of TCGA bladder urothelial carcinoma, somatic FGFR3 G370C was recurrently identified in 3 independent tumor samples, all within the non-T-cell-inflamed subgroup. The authors described G… (full text at CIViC) PMID 27197067 · Sweis et al., 2016 · Open in CIViC	civic
〃	Malignant NeoplasmALIAS	Oncogenic	D	Supports Oncogenicity	3	submitted	EID12843 The extracellular/transmembrane domain mutation G370C was tested in a series of assays for an evaluation of oncogenicity. G370C was stably transfected into 3T3 cells along with WT controls. TAS (Tra… (full text at CIViC) PMID 34272467 · Nakamura et al., 2021 · Open in CIViC	civic
(functional)	—UNRESOLVED	Functional	D	Supports Gain Of Function	2	rejected	EID10414 1 patient's biopsy, which was the common soft subtype of keratinocytic epidermal nevi, displayed a double mutation of both FGFR3 G372C and G382R. The nevus from which the biopsy was taken displayed no… (full text at CIViC) PMID 16841094 · Hafner et al., 2006 · Open in CIViC	civic
〃	—UNRESOLVED	Functional	D	Supports Gain Of Function	2	submitted	EID13141 FGFR3 G370C was functionally evaluated in 293T, L8, and RCJ cells using chemical cross-linking, Western blotting, and c-fos luciferase assays. Compared with WT FGFR3, G370C demonstrated spontaneous li… (full text at CIViC) PMID 12009017 · Adar et al., 2002 · Open in CIViC	civic
Erdafitinib	Transitional Cell Carcinoma	Predictive	A	Supports Sensitivity Response	4	submitted	EID7307 Patients with metastatic or unresectable urothelial carcinoma havoring FGFR2 fusion, FGFR3 fusion or FGFR3 mutation were treated with pan-FGFR inhibitor, erdafitinib, in Phase 2 trial. 96 patients wer… (full text at CIViC) Open in CIViC	civic
〃	Urothelial Carcinoma	Predictive	A	Supports Sensitivity Response	4	submitted	EID13179 In the randomized phase III THOR Cohort 1 trial of patients with advanced or metastatic urothelial carcinoma harboring selected FGFR alterations, FGFR3 G370C was a prespecified qualifying alteration. … (full text at CIViC) PMID 37870920 · Loriot et al., 2023 · Open in CIViC	civic
〃	Urothelial Carcinoma	Predictive	C	Supports Sensitivity Response	3	submitted	EID13176 In a bridging analysis of the phase 2 BLC2001 study of patients with metastatic or locally advanced, surgically unresectable urothelial carcinoma harboring FGFR alterations, 3 erdafitinib-treated pati… (full text at CIViC) PMID 32304281 · Wang et al., 2020 · Open in CIViC	civic
〃	Bladder Urothelial Papillary CarcinomaUNRESOLVED	Predictive	C	Supports Sensitivity Response	3	submitted	EID13180 In the randomized phase II THOR-2 Cohort 1 trial of patients with recurrent, bacillus Calmette-Guérin (BCG)-treated, high-risk, papillary-only non-muscle-invasive bladder cancer harboring select FGFR2… (full text at CIViC) PMID 37871701 · Catto et al., 2024 · Open in CIViC	civic
〃	Malignant NeoplasmALIAS	Predictive	D	Supports Sensitivity Response	3	submitted	EID13175 In a preclinical MANO drug-sensitivity assay, 3T3 cells expressing FGFR3 G370C were treated with erdafitinib for 5 days. Erdafitinib inhibited the viability of FGFR3 G370C-expressing cells with an est… (full text at CIViC) PMID 34272467 · Nakamura et al., 2021 · Open in CIViC	civic
Infigratinib	Transitional Cell Carcinoma	Predictive	B	Supports Sensitivity Response	3	submitted	EID6414 In this one-arm study, 67 patients with metastastic urothelial carcinoma and diverse FGFR3 alterations were treated with pan-FGFR Inhibitor BGJ398. The majority of patients (70.1%) had received two or… (full text at CIViC) PMID 29848605 · Pal et al., 2018 · Open in CIViC	civic
〃	Malignant NeoplasmALIAS	Predictive	D	Supports Sensitivity Response	3	submitted	EID13174 In a preclinical MANO drug-sensitivity assay, 3T3 cells expressing FGFR3 G370C were treated with infigratinib for 5 days. Infigratinib inhibited FGFR3 G370C-expressing cells with an estimated IC50 of … (full text at CIViC) PMID 34272467 · Nakamura et al., 2021 · Open in CIViC	civic
R3Mab	Malignant NeoplasmALIAS	Predictive	D	Does Not Support Resistance	3	accepted	EID10353 Ba/F3 cells transfected with wild type FGFR3 or common bladder carcinoma variant FGFR3 G372C both resulted in IL-3 independence with FGF1 dependence for growth. Monoclonal antibody R3Mab treatment wit… (full text at CIViC) PMID 19381019 · Qing et al., 2009 · Open in CIViC	civic

FGFR3 G380R9
(oncogenic)	Malignant Bladder NeoplasmALIAS	Oncogenic	C	Supports Oncogenicity	1	accepted	EID10490 Targeted mutation analysis on the FGFR3 gene in 297 bladder cancer patients was conducted, where 177 patients were found to harbor FGFR3 mutations. One patient was found to harbor a novel G380R mutati… (full text at CIViC) PMID 12461689 · van Rhijn et al., 2002 · Open in CIViC	civic
(functional)	—UNRESOLVED	Functional	D	Supports Gain Of Function	3	accepted	EID10368 G380R mutation was created in the transmembrane region of murine FGFR3 to study its effect. An in vitro kinase assay using 293 human cells found the mutant FGFR3 to be expressed at levels similar or s… (full text at CIViC) PMID 9136983 · Li et al., 1997 · Open in CIViC	civic
〃	—UNRESOLVED	Functional	D	Supports Gain Of Function	3	submitted	EID10384 In CFK2 chondrocytic cell line model, cells with the G380R variant in FGFR3 showed growth retardation in comparison to the cells expressing the wild type receptor. Cells with the mutant receptor grew … (full text at CIViC) PMID 10646125 · Henderson et al., 2000 · Open in CIViC	civic
〃	—UNRESOLVED	Functional	D	Supports Gain Of Function	3	accepted	EID10396 While receptor tyrosine kinases (RTKs) like FGFR3 form both homodimers and heterodimers (often in the same family), heterodimerization of RTKs is considered to be a method of greater receptor activati… (full text at CIViC) PMID 27927983 · Del Piccolo et al., 2017 · Open in CIViC	civic
〃	—UNRESOLVED	Functional	D	Supports Gain Of Function	3	submitted	EID10403 The G380R variant of FGFR3 was studied in the context of being a known genetic cause for achondroplasia (dwarfism). The authors performed Western blots using HEK 293 cells and antibodies capable of de… (full text at CIViC) PMID 20624921 · He et al., 2010 · Open in CIViC	civic
(oncogenic)	Malignant NeoplasmALIAS	Oncogenic	D	Supports Oncogenicity	3	accepted	EID12847 The transmembrane domain mutation G380R was tested in a series of assays for an evaluation of oncogenicity. G380R was stably transfected into 3T3 cells along with WT controls. TAS (Transformation Ac… (full text at CIViC) PMID 34272467 · Nakamura et al., 2021 · Open in CIViC	civic
(functional)	—UNRESOLVED	Functional	D	Supports Gain Of Function	2	accepted	EID10416 Achondroplasia associated FGFR3 mutant G380R (ACH), K650E (TDII), and wildtype (WT) FGFR3 constructs were cloned and transfected into NIH3T3 cells, and immunopercipitated with anti-FGFR3. In vitro ki… (full text at CIViC) PMID 8754806 · Webster et al., 1996 · Open in CIViC	civic
〃	—UNRESOLVED	Functional	D	Supports Gain Of Function	1	accepted	EID10415 A patient's biopsy, which was the common soft subtype of keratinocytic epidermal nevi, displayed a double mutation of both FGFR3 G372C (aka G370C) and G382R (aka G380R). The nevus from which the biops… (full text at CIViC) PMID 16841094 · Hafner et al., 2006 · Open in CIViC	civic
Infigratinib	Transitional Cell Carcinoma	Predictive	C	Supports Sensitivity Response	2	submitted	EID6413 In this one-arm study, 67 patients with metastastic urothelial carcinoma and diverse FGFR3 alterations were treated with pan-FGFR Inhibitor BGJ398. The majority of patients (70.1%) had received two or… (full text at CIViC) PMID 29848605 · Pal et al., 2018 · Open in CIViC	civic