Treatment of High-Grade Endometrial Stromal Sarcoma and Undifferentiated Uterine Sarcoma

This protocol addresses the management of uterine sarcoma arising specifically as high-grade endometrial stromal sarcoma (HG-ESS) or undifferentiated uterine sarcoma (UUS) — two rare, aggressive histological subtypes with distinct clinical implications.

Clinical scenario

High-grade endometrial stromal sarcoma (HG-ESS) and undifferentiated uterine sarcoma (UUS) collectively represent a small but clinically significant subset of uterine sarcomas. Management decisions are shaped by stage at presentation and whether surgical morcellation occurred.

Treatment approach (partial overview)

For locally advanced disease (stage II–III) or when morcellation has taken place, the approach centres on complete surgical resection followed by post-operative systemic therapy. Additional locoregional treatment may also factor into the plan. Full regimen details, sequencing, and eligibility criteria are in the complete protocol.

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References

HG-ESS and UUS collectively represent about 5% of uterine sarcomas.

In locally advanced stage II and III disease, as well as in cases of morcellation, chemotherapy following complete surgery should be considered.

In cases of morcellation of a HG-ESS or an undifferentiated sarcoma, post-operative chemotherapy should be considered due to the high risk of relapse (IV, B).

Post-operative radiotherapy could be considered based on risks of local recurrence (IV, C).

DOI: 10.1136/ijgc-2024-005823

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