Subtype
Childhood Malignant Kidney Neoplasm
CI-CAN-00002279Explore in graph →
- NCIt
- C123907
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Subtype
CI-CAN-00002279Explore in graph →
Variants & evidence
18 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 |
|---|---|---|---|---|---|---|---|---|
| CCNB3 Fusion3 | ||||||||
| (diagnostic) | Clear Cell Sarcoma of the KidneyALIAS | Diagnostic | C | Supports Positive | 2 | accepted | EID12003In this 2018 study, the authors aimed to identify driver events in 'double negative' clear cell sarcoma of the kidney (CCSK) where neither BCOR ITDs nor the YWHAE::NUTM2 gene fusion are detected. Elev… (full text at CIViC) PMID 28833375 · Wong et al., 2018 · Open in CIViC | civic |
| 〃 | Clear Cell Sarcoma of the KidneyALIAS | Diagnostic | C | Supports Positive | 2 | accepted | EID12004In this 2020 study, the authors report two patients with BCOR::CCNB3 fusion-positive clear cell sarcoma of the kidney (CCSK). Cases were males aged 10 and 14 years who presented with advanced disease.… (full text at CIViC) PMID 31876361 · Han et al., 2020 · Open in CIViC | civic |
| 〃 | Clear Cell Sarcoma of the KidneyALIAS | Diagnostic | C | Supports Positive | 2 | accepted | EID12005In this 2017 study, the authors describe two primary renal sarcomas harboring BCOR::CCNB3 gene fusions and compare the morphological and immunochemical features to typical clear cell sarcoma of the ki… (full text at CIViC) PMID 28817404 · Argani et al., 2017 · Open in CIViC | civic |
| BCOR ITD4 | ||||||||
| (diagnostic) | Clear Cell Sarcoma of the KidneyALIAS | Diagnostic | B | Supports Positive | 5 | accepted | EID10801BCOR internal tandem duplications (ITDs) were found frequently in cases of clear cell sarcoma of kidney (CCSK), a rare pediatric renal cancer. The study initially evaluated 3 CCSK samples by whole exo… (full text at CIViC) PMID 26573325 · Roy et al., 2015 · Open in CIViC | civic |
| (oncogenic) | Clear Cell Sarcoma of the KidneyALIAS | Oncogenic | B | Supports Oncogenicity | 4 | accepted | EID1080227 cases of CCSK were profiled by whole exome sequencing and whole transcriptomics, identifying BCOR ITDs in the majority (23/27, 85%). BCOR encodes a key constituent of a variant polycomb repressive… (full text at CIViC) PMID 26573325 · Roy et al., 2015 · Open in CIViC | civic |
| (diagnostic) | Clear Cell Sarcoma of the KidneyALIAS | |||||||
| EGFR ITD1 | ||||||||
| (diagnostic) | Congenital Mesoblastic Nephroma | Diagnostic | B | Supports Positive | 3 | submitted | EID11281In an analysis of 22 cases of mesoblastic nephroma, 4 of 5 (80%) cellular mesoblastic nephromas harbored ETV6::NTRK3, 1 of 5 cellular mesoblastic nephromas harbored a KLHL7::BRAF fusion, 12 of 12 (100… (full text at CIViC) PMID 32590884 · Zhao et al., 2020 · Open in CIViC | civic |
| NTRK3 Fusion1 | ||||||||
| (diagnostic) | Congenital Mesoblastic Nephroma | Diagnostic | C | Supports Positive | 3 | accepted | EID10420To determine if EML4::NTRK3 fusions were recurrent in tumors known to harbor ETV6::NTRK3 fusions, the authors screened 63 cases with varying diagnoses (infantile fibrosarcoma, congenital mesoblastic n… (full text at CIViC) PMID 29099503 · Church et al., 2018 · Open in CIViC | civic |
| ETV6 ~DEPRECATED-ETV6::NTRK3 FUSION1 | ||||||||
| (diagnostic) | Congenital Mesoblastic Nephroma | Diagnostic | C | Supports Positive | 2 | rejected | EID10417EML6::NTRK3 fusion is seen in cases of Infantile fibrosarcoma and congenital mesoblastic nephroma. In general, these types of tumors more commonly harbor ETV6::NTRK3 fusions. PMID 29099503 · Church et al., 2018 · Open in CIViC | civic |
| NTRK3 Fusion5 | ||||||||
| (diagnostic) | Congenital Mesoblastic Nephroma | Diagnostic | B | Supports Positive | 3 | accepted | EID10841A frequent genetic alteration in congenital mesoblastic nephroma is the translocation t(12;15) resulting in a fusion of the ETV6 gene on 12p13 and the NTRK3 gene on 15p15 that occurs almost exclusivel… (full text at CIViC) PMID 29286563 · Vokuhl et al., 2018 · Open in CIViC | civic |
| (prognostic) | Congenital Mesoblastic Nephroma | Prognostic | B | Supports Better Outcome | 3 | submitted | EID10842In a group of 111 mesoblastic nephroma (MN) samples, 79 were were evaluable by FISH, which consisted of 38 classical MN, 29 cellular MN, and 12 mixed MN samples. 5 year relapse free survival (RFS) was… (full text at CIViC) PMID 29286563 · Vokuhl et al., 2018 · Open in CIViC | civic |
| (diagnostic) | Congenital Mesoblastic Nephroma | Diagnostic | B | Supports Positive | 3 | |||
| NUTM2A Fusion + NUTM2B Fusion + NUTM2E FusionNUTM2ANUTM2BNUTM2E3 | ||||||||
| (diagnostic) | Clear Cell Sarcoma of the KidneyALIAS | Diagnostic | B | Supports Positive | 3 | accepted | EID11782In this 2012 study O’Meara et al. included 51 clear cell sarcoma of kidney (CCSK). Using an index CCSK with the t(10;17)(q22;p13) identified by cytogenetics, they were able to map the breakpoints and … (full text at CIViC) PMID 22294382 · O'Meara et al., 2012 · Open in CIViC | civic |
| 〃 | Clear Cell Sarcoma of the KidneyALIAS | Diagnostic | C | Supports Positive | 2 | accepted | EID11783In this 2020 study Kenny et al. included 68 cases of clear cell sarcoma of the kidney (CCSK). Using RT-PCR with RNA extracted from FFPE tumour tissue they detected a YWHAE::NUTM2 fusion transcript in… (full text at CIViC) PMID 32364435 · Kenny et al., 2020 · Open in CIViC | |
Data updated 14 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.
| Diagnostic |
| B |
| Supports Positive |
| 4 |
| accepted |
EID11423High BCOR expression which was associated with an internal tandem duplication (ITD) of 89 to 144 bp in exon 16 of the BCOR gene was identified in 20 of 20 (100%) of cases of clear cell sarcoma of the … (full text at CIViC) PMID 26098867 · Ueno-Yokohata et al., 2015 · Open in CIViC |
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| 〃 | Clear Cell Sarcoma of the KidneyALIAS | Diagnostic | B | Supports Positive | 3 | accepted | EID11424Whole transcriptome sequencing on tumors from 8 patients with clear cell sarcoma of the kidney identified BCOR ITD and overexpression of BCOR in all samples. The BCOR ITD was confirmed on tumor DNA.… (full text at CIViC) PMID 26516930 · Astolfi et al., 2015 · Open in CIViC | civic |
| accepted |
EID11279In an analysis of 19 cases of mesoblastic nephroma, the ETV6::NTRK3 fusion was detected in 0 of 5 of the classic mesoblastic nephroma, 8 of 8 (100%) of the cellular mesoblastic nephroma and 5 of 6 (83… (full text at CIViC) PMID 27020209 · El Demellawy et al., 2016 · Open in CIViC |
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| 〃 | Congenital Mesoblastic Nephroma | Diagnostic | B | Supports Positive | 2 | accepted | EID11280In an analysis of 22 cases of mesoblastic nephroma, 4 of 5 (80%) cellular mesoblastic nephromas harbored ETV6::NTRK3, 1 of 5 cellular mesoblastic nephromas harbored a KLHL7::BRAF fusion, 12 of 12 (100… (full text at CIViC) PMID 32590884 · Zhao et al., 2020 · Open in CIViC | civic |
| Larotrectinib | Congenital Mesoblastic Nephroma | Predictive | C | Supports Sensitivity Response | 4 | accepted | EID10840A 23 month old (at the time of report) male was diagnosed prenatally with Stage II cellular mesoblastic nephroma (MN) on the right kidney, which was surgically removed on the sixth day of life. ETV6::… (full text at CIViC) PMID 29893456 · Halalsheh et al., 2018 · Open in CIViC | civic |
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| 〃 | Clear Cell Sarcoma of the KidneyALIAS | Diagnostic | C | Supports Positive | 2 | accepted | EID11784In this 2016 study Kenny et al. included 159 cases of clear cell sarcoma of the kidney (CCSK). RNA was extracted from either snap-frozen or FFPE tumour tissue samples and real-time PCR used to identif… (full text at CIViC) PMID 27000436 · Kenny et al., 2016 · Open in CIViC | civic |