WT1 Fusion

CI-VAR-00001282Explore in graph →CIViC 2941

Curated evidence

Evidence by cancer (5 items)

Grouped by cancer context first, then therapy. The same variant can be sensitizing in one cancer and irrelevant in another — contexts are never merged. 50 items per page; a cancer group may continue on the next page.

civicCuratedShowing 1–5 of 5 evidence items across 3 cancer contexts on this page · levels, directions and significance as curated at the source; each row links to its CIViC record.

Molecular profile	Therapy	Type	Level	Direction · significance	Rating (1–5)	Status	Evidence	Source
Desmoplastic Small Round Cell Tumor3
EWSR1::WT1 Fusion	(diagnostic)	Diagnostic	B	Supports Positive	2	accepted	EID11405 In an analysis of a cohort of desmoplastic small round cell tumor, evidence of a functional EWSR1::WT1 fusion was detected in 25 of 26 cases. In these cases, the genomic breakpoints in WT1 was in in… (full text at CIViC) PMID 9738572 · Gerald et al., 1998 · Open in CIViC	civic
EWSR1::WT1 Fusion	(diagnostic)	Diagnostic	B	Supports Positive	2	accepted	EID11406 In an analysis of 6 desmoplastic small round cell tumors, chimeric EWSR1::WT1 transcripts were detected in 4. The translocation resulted in an in-frame fusion of the N terminus (first 7 exons) of EWS… (full text at CIViC) PMID 7862627 · Gerald et al., 1995 · Open in CIViC	civic

ClinVar

Clinical significance (0)

ClinVar interpretations are shown as structured records — significance, review status, star rating, conditions — never flattened into one word.

Data not yet available

No ClinVar interpretation attached to this variant.

Sarcoma1
EWSR1::FLI1 Fusion OR EWSR1::WT1 Fusion OR EWSR1::ATF1 Fusion	Trabectedin	Predictive	B	Supports Sensitivity Response	2	submitted	EID12544 In a pooled analysis of two phase II studies (n = 66) of advanced translocation-related sarcoma, trabectedin (1.2 mg/m² q3w) produced median PFS 5.6 months (95% CI 4.1–7.3) and median OS 17.5 months (… (full text at CIViC) PMID 28526720 · Takahashi et al., 2017 · Open in CIViC	civic

Unmapped disease1unmapped disease
EWSR1::WT1 Fusion	(functional)	Functional	D	Supports Gain Of Function	3	submitted	EID7921 To find authentic targets regulated by EWSR1-WT1 fusion protein in desmoplastic small round-cell tumor (DSRT), a primitive tumor characterized by t(11;22)(p13;q12) chromosomal translocation, the autho… (full text at CIViC) PMID 9354795 · Lee et al., 1997 · Open in CIViC	civic