ArticleClinical cancer research : an official journal of the American Association for Cancer Research2010
Subtype-specific FBXW7 mutation and MYCN copy number gain in Wilms' tumor.
Article in Clinical cancer research : an official journal of the American Association for Cancer Research, 2010. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT00047138 (Nephroblastoma), which is not on this map. Cited by 40 papers, 2 of them syntheses that pooled it.
What it found
Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.
The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Nephroblastoma (Wilms Tumour) Clinical Trial And Study
Who cites it
40 citing papers in PubMed, 2 syntheses or guidelines pooled it, 82 citations in OpenAlex.
- Systematic Review: Prognostic Molecular Biomarkers in Wilms Tumors.JCO precision oncology · 2026Pooled it
- Genetic variation frequencies in Wilms' tumor: A meta-analysis and systematic review.Cancer science · 2016Pooled it
- Gain of 1q As a Prognostic Biomarker in Wilms Tumors (WTs) Treated With Preoperative Chemotherapy in the International Society of Paediatric Oncology (SIOP) WT 2001 Trial: A SIOP Renal Tumours Biology Consortium Study.Journal of clinical oncology : official journal of the American Society of Clinical Oncology · 2016Trial
- Definition of parameters and thresholds to detect MYCN amplification in retinoblastomas.Virchows Archiv : an international journal of pathology · 2026Article
- Improving the diagnosis of renal tumours of young people through integrated molecular analysis.Journal of cancer research and clinical oncology · 2026Article
- Disruption of the KLHL37-N-Myc complex restores N-Myc degradation and arrests neuroblastoma growth in mouse models.The Journal of clinical investigation · 2025Article
- Gene Expression Analysis of (Paired) Primary and Relapsed Wilms Tumor Samples to Unravel the Underlying Factors Driving Tumor Recurrence.Cancer medicine · 2025Article
- Incidence and Survival of Children and Adolescents With Wilms Tumor, United States, 2001-2020.Cancer medicine · 2025Article
- Roles of E3 ubiquitin ligases and deubiquitinating enzymes in renal cell carcinoma.Frontiers in oncology · 2025Review
- MYCN as an oncogene in pediatric brain tumors.Frontiers in oncology · 2025Review
- Hallmark discoveries in the biology of Wilms tumour.Nature reviews. Urology · 2024Review
- Review
- Review
- Differential expression profiling of onco and tumor-suppressor genes from major-signaling pathways in Wilms' tumor.Pediatric surgery international · 2022Article
- Investigating the role of somatic sequencing platforms for phaeochromocytoma and paraganglioma in a large UK cohort.Clinical endocrinology · 2022Review
- Genetic changes associated with relapse in favorable histology Wilms tumor: A Children's Oncology Group AREN03B2 study.Cell reports. Medicine · 2022Article
- A novel combination therapy targeting ubiquitin-specific protease 5 in MYCN-driven neuroblastoma.Oncogene · 2021Article
- Article
- Article
- MYCN gene polymorphisms and Wilms tumor susceptibility in Chinese children.Journal of clinical laboratory analysis · 2019Article
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
16 authors at 4 institutions in 2 countries.
Funding
Abstract
purposeWilms' tumor (WT), the most common pediatric renal malignancy, is associated with mutations in several well-characterized genes, most notably WT1, CTNNB1, WTX, and TP53. However, the majority of cases do not harbor mutations in these genes. We hypothesized that additional drivers of tumor behavior would be contained within areas of consistent genomic copy number change, especially those associated with the WT risk groups defined by the International Society of Paediatric Oncology (SIOP). EXPERIMENTAL
designWe analyzed high-resolution (Affymetrix 250K single nucleotide polymorphism array) genomic copy number profiles of over 100 tumors from selected risk groups treated under the SIOP protocols, further characterizing genes of interest by sequencing, Multiplex Ligation-dependent Probe Amplification, or fluorescence in situ hybridization.
resultsWe identified FBXW7, an E3 ubiquitin ligase component, as a novel Wilms' tumor gene, mutated or deleted in approximately 4% of tumors examined. Strikingly, 3 of 14 (21%) of tumors with epithelial type histology after neoadjuvant chemotherapy had FBXW7 aberrations, whereas a fourth WT patient had germline mutations in both FBXW7 and WT1. We also showed that MYCN copy number gain, detected in 9 of 104 (8.7%) of cases, is relatively common in WT and significantly more so in tumors of the high risk diffuse anaplastic subtype (6 of 19, 32%).
conclusionsBecause MYCN is itself a target of FBXW7-mediated ubiquitination and degradation, these results suggest that a common pathway is dysregulated by different mechanisms in various WT subtypes. Emerging therapies that target MYCN, which is amplified in several other pediatric cancers, may therefore be of value in high risk Wilms' tumor.
Indexed as
Identifiers
What OpenQuestion holds
Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.