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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

Back to Ovarian Rhabdomyosarcoma overview
Ovarian Rhabdomyosarcoma: Alveolar (PAX–FOXO1+ subset)
Ovarian Rhabdomyosarcoma: Spindle cell / sclerosing
Ovarian Rhabdomyosarcoma: Pleomorphic (adult)

Educational use only; not medical advice.