Ovarian Rhabdomyosarcoma: Alveolar (PAX–FOXO1+ subset)

Last reviewed: 9/15/2025

Applies to: Alveolar histology with or without confirmed PAX–FOXO1 fusion.

Summary

Fusion-driven subset with higher nodal propensity and worse outcomes; requires aggressive multimodality care.

Pathology & Markers

  • PAX3/7–FOXO1 fusion by FISH/NGS in many cases
  • Strong myogenin/MyoD1 nuclear staining

Biology & Pathways

  • PAX–FOXO1 transcriptional program
  • IGF/PI3K/AKT dependence

Clinical Challenges

  • Higher risk of nodal and hematogenous spread
  • Potential chemoresistance; consider trials early

Imaging Notes

  • Chest CT essential; consider PET/CT for staging

Management Modifiers

Related

Educational use only; not medical advice.