Gene

TFE3

transcription factor binding to IGHM enhancer 3

Explore in graph →CI-GENE-00034997HGNC:11752 ENSG00000068323 NCBI 7030 Cancer geneApproved

Curated evidence

Clinical evidence (22)

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

civicCuratedShowing 1–22 of 22 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
TFE3 Fusion7
(diagnostic)	Alveolar Soft Part Sarcoma	Diagnostic	B	Supports Positive	4	accepted	EID9978 Alveolar soft part sarcoma (ASPS) is characterized by the presence of an unbalanced translocation der(17)t(X;17)(p11;q25). In this 2001 paper, FISH with YAC and cosmid probes was used to localize the…

Cohorts

Alteration frequency by cohort (0)

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

Data not yet available

No cohort frequency recorded for this gene.

Literature

Linked publications (14)

25 per page, newest first.

pubmedPublishedShowing 1–14 of 14 publications · bibliographic data as recorded by PubMed; abstracts are not reproduced.

The der(17)t(X;17)(p11;q25) of human alveolar soft part sarcoma fuses the TFE3 transcription factor gene to ASPL, a novel gene at 17q25.
· Oncogene · 2001 · PMID 11244503 · validated civic_curation pubmed
Renal cell carcinoma associated with Xp11.2 translocation/TFE3 gene fusions: clinical experience and literature review.
· Future Oncol · 2015 · PMID 26169997 · validated civic_curation pubmed
Technique for differentiating alveolar soft part sarcoma from other tumors in paraffin-embedded tissue: comparison of immunohistochemistry for TFE3 and CD147 and of reverse transcription polymerase chain reaction for ASPSCR1-TFE3 fusion transcript.
· Hum Pathol · 2012 · PMID 21835426 · validated civic_curation pubmed
TFE3-Fusion Variant Analysis Defines Specific Clinicopathologic Associations Among Xp11 Translocation Cancers.

TFE3 VCP::TFE31
(prognostic)	Renal Cell CarcinomaCURATED_BROADER	Prognostic	B	Supports Poor Outcome	4	rejected	EID10148 This study evaluates 200 nephrectomy cases. Within the 200 cases, there are four cases that are Xp11.2 translocation renal cell carcinomas (RCC). Previous studies identified two stages (pediatric and … (full text at CIViC) PMID 34917511 · Ge et al., 2021 · Open in CIViC	civic

TFE3 VCP::TFE31

(prognostic)

Renal Cell CarcinomaCURATED_BROADER

Prognostic

Supports Poor Outcome

rejected

EID10148

This study evaluates 200 nephrectomy cases. Within the 200 cases, there are four cases that are Xp11.2 translocation renal cell carcinomas (RCC). Previous studies identified two stages (pediatric and … (full text at CIViC)

PMID 34917511 · Ge et al., 2021 · Open in CIViC

civic

TFE3 Fusion1
(prognostic)	Renal Cell CarcinomaCURATED_BROADER	Prognostic	C	Supports Better Outcome	1	submitted	EID12392 A case of metastatic renal cell carcinoma (RCC) is reported with a novel translocation t(X;9)(p11.23;p13.3) and RNA-seq identification of a putative corresponding fusion: VCP::TFE3. TFE3 fusion positi… (full text at CIViC) PMID 36695651 · Kuentz et al., 2023 · Open in CIViC	civic

TFE3 Fusion1

(prognostic)

Renal Cell CarcinomaCURATED_BROADER

Prognostic

Supports Better Outcome

submitted

EID12392

A case of metastatic renal cell carcinoma (RCC) is reported with a novel translocation t(X;9)(p11.23;p13.3) and RNA-seq identification of a putative corresponding fusion: VCP::TFE3. TFE3 fusion positi… (full text at CIViC)

PMID 36695651 · Kuentz et al., 2023 · Open in CIViC

civic

TFE3 Fusion9
(diagnostic)	Renal Cell Carcinoma with MiT Translocations	Diagnostic	B	Supports Positive	4	accepted	EID10150 This study screened 983 Renal Cell Carcinoma (RCC) cases and identified 65 tumors with morphologic features suspicious of RCC with Xp11.2 translocation/TFE3 gene fusion (Xp11.2 RCC). TFE3 immunohistoc… (full text at CIViC) PMID 26169997 · He et al., 2015 · Open in CIViC	civic
〃	TFE3-Rearranged Renal Cell Carcinoma	Diagnostic	B	Supports Positive	4	accepted	EID11533 Argani. et al,. (2016) included a total of 60 cases with known TFE3 gene rearrangements in this study of Xp11 translocation positive neoplasms (47 RCC, 8 PEComa, and 5 melanotic renal cancers). The ag… (full text at CIViC) PMID 26975036 · Argani et al., 2016 · Open in CIViC	civic
〃	PEComaALIAS	Diagnostic	B	Supports Positive	4	accepted	EID11606 In this study, the authors evaluated 60 Xp11 translocation neoplasms, including 8 perivascular epithelioid cell tumors (PEComas), to characterize the diagnostic significance of TFE3 fusion variants. U… (full text at CIViC) PMID 26975036 · Argani et al., 2016 · Open in CIViC	civic
〃	Renal Cell Carcinoma with MiT Translocations	Diagnostic	B	Supports Positive	4	accepted	EID11701 Bakouny, et al. (2022) performed a comprehensive study of the molecular and clinical features of 90 translocation renal cell carcinomas (tRCCs), defined as subtype of kidney cancer driven by MiT/TFE g… (full text at CIViC) PMID 34986355 · Bakouny et al., 2022 · Open in CIViC	civic
〃	PEComaALIAS	Diagnostic	B	Supports Positive	3	accepted	EID12690 In this study the authors examined six gynecologic tract perivascular epithelioid cell tumors (PEComas) that were previously diagnosed as PEComa but demonstrated unusual morphology suggestive of TFE3 … (full text at CIViC) PMID 25517951 · Schoolmeester et al., 2015 · Open in CIViC	civic
〃	PEComaALIAS	Diagnostic	B	Supports Positive	3	accepted	EID12691 In this 2025 study, the authors included 14 confirmed TFE3-rearranged perivascular epithelioid cell tumors (PEComas), previously classified as benign (9 tumors) or malignant (5 tumors), and compared t… (full text at CIViC) PMID 40462401 · Papke et al., 2025 · Open in CIViC	civic
(prognostic)	Renal Cell Carcinoma with MiT Translocations	Prognostic	B	Supports Better Outcome	2	submitted	EID10164 This study screened 983 Renal Cell Carcinoma (RCC) cases and identified 65 tumors with morphologic features suspicious of RCC with Xp11.2 translocation/TFE3 gene fusion (Xp11.2 RCC). TFE3 immunohistoc… (full text at CIViC) PMID 26169997 · He et al., 2015 · Open in CIViC	civic
〃	Renal Cell Carcinoma with MiT Translocations	Prognostic	C	Supports Poor Outcome	4	submitted	EID10149 This study evaluates 200 nephrectomy cases. Within the 200 cases, there are four cases that are Xp11.2 translocation renal cell carcinomas (RCC). Previous studies identified two stages (pediatric and … (full text at CIViC) PMID 34917511 · Ge et al., 2021 · Open in CIViC	civic
(diagnostic)	Renal Cell Carcinoma with MiT Translocations	Diagnostic	C	Supports Positive	2	accepted	EID11683 A study of 443 renal cell carcinoma identified 7 cases with an Xp11 translocation. The median age of the 7 patients was 41 years (range, 15 to 59 years). Of the 244 cases with a successful karyotype, … (full text at CIViC) PMID 19228722 · Komai et al., 2009 · Open in CIViC	civic

TFE3 Fusion9

(diagnostic)

Renal Cell Carcinoma with MiT Translocations

Diagnostic

Supports Positive

accepted

EID10150

This study screened 983 Renal Cell Carcinoma (RCC) cases and identified 65 tumors with morphologic features suspicious of RCC with Xp11.2 translocation/TFE3 gene fusion (Xp11.2 RCC). TFE3 immunohistoc… (full text at CIViC)

PMID 26169997 · He et al., 2015 · Open in CIViC

civic

〃

TFE3-Rearranged Renal Cell Carcinoma

Diagnostic

Supports Positive

accepted

EID11533

Argani. et al,. (2016) included a total of 60 cases with known TFE3 gene rearrangements in this study of Xp11 translocation positive neoplasms (47 RCC, 8 PEComa, and 5 melanotic renal cancers). The ag… (full text at CIViC)

PMID 26975036 · Argani et al., 2016 · Open in CIViC

civic

〃

PEComaALIAS

Diagnostic

Supports Positive

accepted

EID11606

In this study, the authors evaluated 60 Xp11 translocation neoplasms, including 8 perivascular epithelioid cell tumors (PEComas), to characterize the diagnostic significance of TFE3 fusion variants. U… (full text at CIViC)

PMID 26975036 · Argani et al., 2016 · Open in CIViC

civic

〃

Renal Cell Carcinoma with MiT Translocations

Diagnostic

Supports Positive

accepted

EID11701

Bakouny, et al. (2022) performed a comprehensive study of the molecular and clinical features of 90 translocation renal cell carcinomas (tRCCs), defined as subtype of kidney cancer driven by MiT/TFE g… (full text at CIViC)

PMID 34986355 · Bakouny et al., 2022 · Open in CIViC

civic

〃

PEComaALIAS

Diagnostic

Supports Positive

accepted

EID12690

In this study the authors examined six gynecologic tract perivascular epithelioid cell tumors (PEComas) that were previously diagnosed as PEComa but demonstrated unusual morphology suggestive of TFE3 … (full text at CIViC)

PMID 25517951 · Schoolmeester et al., 2015 · Open in CIViC

civic

〃

PEComaALIAS

Diagnostic

Supports Positive

accepted

EID12691

In this 2025 study, the authors included 14 confirmed TFE3-rearranged perivascular epithelioid cell tumors (PEComas), previously classified as benign (9 tumors) or malignant (5 tumors), and compared t… (full text at CIViC)

PMID 40462401 · Papke et al., 2025 · Open in CIViC

civic

(prognostic)

Renal Cell Carcinoma with MiT Translocations

Prognostic

Supports Better Outcome

submitted

EID10164

PMID 26169997 · He et al., 2015 · Open in CIViC

civic

〃

Renal Cell Carcinoma with MiT Translocations

Prognostic

Supports Poor Outcome

submitted

EID10149

PMID 34917511 · Ge et al., 2021 · Open in CIViC

civic

(diagnostic)

Renal Cell Carcinoma with MiT Translocations

Diagnostic

Supports Positive

accepted

EID11683

A study of 443 renal cell carcinoma identified 7 cases with an Xp11 translocation. The median age of the 7 patients was 41 years (range, 15 to 59 years). Of the 244 cases with a successful karyotype, … (full text at CIViC)

PMID 19228722 · Komai et al., 2009 · Open in CIViC

civic

TFE3 Fusion4
(diagnostic)	Malignant Epithelioid HemangioendotheliomaUNRESOLVED	Diagnostic	B	Supports Positive	4	accepted	EID11682 This 2021 paper by Dermawan, et al. was a multi-institutional clinicopathological study of 24 cases of YAP1::TFE3 fusion-positive epithelioid hemangioendotheliomas (EHE), with an age range of 20 to 78… (full text at CIViC) PMID 34381186 · Dermawan et al., 2021 · Open in CIViC	civic
〃	Malignant Epithelioid HemangioendotheliomaUNRESOLVED	Diagnostic	B	Supports Positive	3	accepted	EID11536 This study by Antonescu, et al. (2013) included 10 cases with a diagnosis of epithelioid hemangioendothelioma (EHE), which showed unusual morphologic features such as abundant eosinophilic cytoplasm, … (full text at CIViC) PMID 23737213 · Antonescu et al., 2013 · Open in CIViC	civic
(oncogenic)	Malignant Epithelioid HemangioendotheliomaUNRESOLVED	Oncogenic	B	Supports Oncogenicity	2	accepted	EID11538 This 2021 paper by Dermawan, et al. included 24 cases of YAP1::TFE3 fusion-positive epithelioid hemangioendotheliomas (EHE), with an age range of 20 to 78 years old. All cases were confirmed by molecu… (full text at CIViC) PMID 34381186 · Dermawan et al., 2021 · Open in CIViC	civic
(diagnostic)	Malignant Epithelioid HemangioendotheliomaUNRESOLVED	Diagnostic	C	Supports Positive	2	accepted	EID11537 This 2023 study included 29 epithelioid hemangioendothelioma (EHE) cases, with the median patient age of 53 years. Eleven of the 29 tumors had a targeted sequencing panel which identified the WWTR1::C… (full text at CIViC) PMID 37810439 · Lahori et al., 2023 · Open in CIViC	civic

TFE3 Fusion4

(diagnostic)

Malignant Epithelioid HemangioendotheliomaUNRESOLVED

Diagnostic

Supports Positive

accepted

EID11682

This 2021 paper by Dermawan, et al. was a multi-institutional clinicopathological study of 24 cases of YAP1::TFE3 fusion-positive epithelioid hemangioendotheliomas (EHE), with an age range of 20 to 78… (full text at CIViC)

PMID 34381186 · Dermawan et al., 2021 · Open in CIViC

civic

〃

Malignant Epithelioid HemangioendotheliomaUNRESOLVED

Diagnostic

Supports Positive

accepted

EID11536

This study by Antonescu, et al. (2013) included 10 cases with a diagnosis of epithelioid hemangioendothelioma (EHE), which showed unusual morphologic features such as abundant eosinophilic cytoplasm, … (full text at CIViC)

PMID 23737213 · Antonescu et al., 2013 · Open in CIViC

civic

(oncogenic)

Malignant Epithelioid HemangioendotheliomaUNRESOLVED

Oncogenic

Supports Oncogenicity

accepted

EID11538

This 2021 paper by Dermawan, et al. included 24 cases of YAP1::TFE3 fusion-positive epithelioid hemangioendotheliomas (EHE), with an age range of 20 to 78 years old. All cases were confirmed by molecu… (full text at CIViC)

PMID 34381186 · Dermawan et al., 2021 · Open in CIViC

civic

(diagnostic)

Malignant Epithelioid HemangioendotheliomaUNRESOLVED

Diagnostic

Supports Positive

accepted

EID11537

This 2023 study included 29 epithelioid hemangioendothelioma (EHE) cases, with the median patient age of 53 years. Eleven of the 29 tumors had a targeted sequencing panel which identified the WWTR1::C… (full text at CIViC)

PMID 37810439 · Lahori et al., 2023 · Open in CIViC

civic