Detection of the PAX3-FKHR fusion gene in paediatric rhabdomyosarcoma: a reproducible predictor of outcome?
2001

Predicting Outcomes in Rhabdomyosarcoma with PAX3-FKHR Gene

Sample size: 91 publication Evidence: moderate

Author Information

Author(s): Anderson J, Gordon T, McManus A, Mapp T, Gould S, Kelsey A, McDowell H, Pinkerton R, Shipley J, Pritchard-Jones K

Primary Institution: Institute of Cancer Research, Sutton, Surrey, UK

Hypothesis

Is the PAX3-FKHR fusion gene a reliable predictor of outcome in paediatric rhabdomyosarcoma?

Conclusion

The presence of the PAX3-FKHR translocation is an adverse prognostic factor in rhabdomyosarcoma.

Supporting Evidence

  • The study found that 82% of alveolar cases had one of the characteristic translocations.
  • Univariate survival analysis indicated that the PAX3-FKHR translocation is an adverse prognostic factor.
  • The variant PAX7-FKHR translocation occurs in younger patients with more localized disease.

Takeaway

Doctors can look for a specific gene in cancer cells to help decide how to treat kids with a type of muscle cancer.

Methodology

The study used classical cytogenetics, FISH, and RT-PCR to analyze 91 primary rhabdomyosarcoma tumors.

Limitations

The hypothesis requires testing in a prospective study.

Participant Demographics

The study included patients with varying ages and disease characteristics.

Statistical Information

P-Value

p<0.05

Statistical Significance

p<0.05

Digital Object Identifier (DOI)

10.1054/bjoc.2001.2008

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