Ovarian Rhabdomyosarcoma: Embryonal

Last reviewed: 9/15/2025

Applies to: Embryonal histology in the ovary.

Summary

Most reported gynecologic RMS subtype; may present in AYA. Managed with surgical resection plus VAC-based chemotherapy; RT as indicated.

Pathology & Markers

  • Desmin+, myogenin+, MyoD1+
  • Usually fusion-negative for PAX–FOXO1

Biology & Pathways

  • Myogenic program activation
  • PI3K/AKT/mTOR signaling

Clinical Challenges

  • Mass effect symptoms and risk of torsion/rupture
  • Need for fertility counseling in AYA

Imaging Notes

  • CT CAP baseline; MRI pelvis for local mapping

Management Modifiers

Related

Educational use only; not medical advice.