Prognostic value of PAX3/7-FOXO1 fusion status in alveolar rhabdomyosarcoma: Systematic review and meta-analysis

Crit Rev Oncol Hematol. 2015 Oct;96(1):46-53. doi: 10.1016/j.critrevonc.2015.04.012. Epub 2015 May 14.

Abstract

Purpose: The objective of this systematic review is to provide an unprecedented summary of the prognostic impact of PAX3/7-FOXO1 fusion status in alveolar rhabdomyosarcoma.

Methods: Studies evaluating PAX3/7-FOXO1 fusion gene or its variants as a prognostic marker were systematically searched and comparative meta-analysis of overall survival was carried out.

Results: A total of 7 studies comprising 993 patients with rhabdomyosarcoma were included. Three eligible studies showed no significant difference of survival between fusion positive and negative alveolar rhabdomyosarcoma. Four eligible studies showed that PAX3-FOXO1 fusion variant may indicate a lower survival probability than PAX7-FOXO1, although the effect did not reach a level of statistical significance (pooled hazard ratios, 1.66; 95% CI, 0.95-2.89; p=0.07).

Conclusions: There was no significant difference in the overall survival between patients with the positive and negative fusion gene, but there were indications of PAX3-FOXO1 being an unfavorable prognostic factor.

Keywords: Fusion gene; Meta-analysis; PAX3/7–FOXO1; Rhabdomyosarcoma; Survival.

Publication types

  • Meta-Analysis
  • Review
  • Systematic Review

MeSH terms

  • Forkhead Box Protein O1 / genetics*
  • Gene Fusion*
  • Humans
  • PAX3 Transcription Factor / genetics*
  • PAX7 Transcription Factor / genetics*
  • Prognosis
  • Proportional Hazards Models
  • Rhabdomyosarcoma, Alveolar / genetics*
  • Rhabdomyosarcoma, Alveolar / mortality

Substances

  • Forkhead Box Protein O1
  • PAX3 Transcription Factor
  • PAX3 protein, human
  • PAX7 Transcription Factor
  • PAX7 protein, human