ArticleBritish journal of cancer2001
Detection of the PAX3-FKHR fusion gene in paediatric rhabdomyosarcoma: a reproducible predictor of outcome?
Article in British journal of cancer, 2001. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 28 papers, 1 of them a synthesis that pooled it.
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Who cites it
28 citing papers in PubMed, 1 synthesis or guideline pooled it, 107 citations in OpenAlex.
- Gene translocations in musculoskeletal neoplasms.Clinical orthopaedics and related research · 2008Pooled it
- PAX3 haploinsufficiency in Maine Coon cats with dominant blue eyes and hearing loss resembling the human Waardenburg syndrome.G3 (Bethesda, Md.) · 2024Article
- An update on rhabdomyosarcoma risk stratification and the rationale for current and future Children's Oncology Group clinical trials.Pediatric blood & cancer · 2022Review
- Improving Risk Stratification for Pediatric Patients with Rhabdomyosarcoma by Molecular Detection of Disseminated Disease.Clinical cancer research : an official journal of the American Association for Cancer Research · 2021Article
- Article
- Oncogenic role of HMGA2 in fusion-negative rhabdomyosarcoma cells.Cancer cell international · 2020Article
- Intratumoral Translocation Positive Heterogeneity in Pediatric Alveolar Rhabdomyosarcoma Tumors Correlates to Patient Survival Prognosis.Frontiers in cell and developmental biology · 2020Article
- OLIG2 is a novel immunohistochemical marker associated with the presence of PAX3/7-FOXO1 translocation in rhabdomyosarcomas.Diagnostic pathology · 2019Article
- A Perspective on Polo-Like Kinase-1 Inhibition for the Treatment of Rhabdomyosarcomas.Frontiers in oncology · 2019Review
- Translocation-Related Sarcomas.International journal of molecular sciences · 2018Review
- MicroRNA and gene co-expression networks characterize biological and clinical behavior of rhabdomyosarcomas.Cancer letters · 2017Article
- Genetics of Bladder Malignant Tumors in Childhood.Current genomics · 2016Article
- Clinical Application of Prognostic Gene Expression Signature in Fusion Gene-Negative Rhabdomyosarcoma: A Report from the Children's Oncology Group.Clinical cancer research : an official journal of the American Association for Cancer Research · 2015Article
- Identification of target genes of PAX3-FOXO1 in alveolar rhabdomyosarcoma.Oncology reports · 2013Article
- The inconvenience of convenience cohorts: rhabdomyosarcoma and the PAX-FOXO1 biomarker.Cancer epidemiology, biomarkers & prevention : a publication of the American Association for Cancer Research, cosponsored by the American Society of Preventive Oncology · 2012Review
- New miRNA labeling method for bead-based quantification.BMC molecular biology · 2010Article
- Credentialing a preclinical mouse model of alveolar rhabdomyosarcoma.Cancer research · 2009Article
- PAX3-FOXO1 fusion gene in rhabdomyosarcoma.Cancer letters · 2008Review
- Rhabdomyosarcoma subtyping by immunohistochemical assessment of myogenin: tissue array study and review of the literature.Pathology oncology research : POR · 2008Review
- Clinical relevance of molecular genetics to paediatric sarcomas.Journal of clinical pathology · 2007Review
Corrections and comments
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Authors and funding
11 authors at 3 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Rhabdomyosarcoma has 2 major histological subtypes, embryonal and alveolar. Alveolar histology is associated with the fusion genes PAX3-FKHR and PAX7-FKHR. Definition of alveolar has been complicated by changes in terminology and subjectivity. It is currently unclear whether adverse clinical behaviour is better predicted by the presence of these fusion genes or by alveolar histology. We have determined the presence of the PAX3/7-FKHR fusion genes in 91 primary rhabdomyosarcoma tumours using a combination of classical cytogenetics, FISH and RT-PCR, with a view to determining the clinical characteristics of tumours with and without the characteristic translocations. There were 37 patients with t(2;13)/PAX3-FKHR, 8 with t(1;13) PAX7-FKHR and 46 with neither translocation. One or other of the characteristic translocations was found in 31/38 (82%) of alveolar cases. Univariate survival analysis revealed the presence of the translocation t(2;13)/PAX3-FKHR to be an adverse prognostic factor. With the difficulties in morphological diagnosis of alveolar rhabdomyosarcoma on increasingly used small needle biopsy specimens, these data suggest that molecular analysis for PAX3-FKHR will be a clinically useful tool in treatment stratification in the future. This hypothesis requires testing in a prospective study. Variant t(1;13)/PAX7-FKHR appears biologically different, occurring in younger patients with more localised disease.
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