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Endometrial Stromal Sarcoma: An Update
Giulio Ricotta1, Silvio Andrea Russo1,2, Anna Fagotti2
1Surgical Oncology, Oncopole Claudius Regaud-Institut Universitaire du Cancer Toulouse Oncopole, 31100 Toulouse, France.
Cancers
|June 13, 2025
Summary
Endometrial stromal sarcoma (ESS) is a rare uterine cancer with low-grade (LG-ESS) and high-grade (HG-ESS) subtypes. Treatment and prognosis vary significantly, with LG-ESS responding to hormonal therapy and HG-ESS requiring chemotherapy.
Area of Science:
- Gynecologic Oncology
- Pathology
- Medical Genetics
Background:
- Endometrial stromal sarcoma (ESS) is a rare uterine malignancy comprising 15-20% of uterine sarcomas.
- ESS is categorized into low-grade (LG-ESS) and high-grade (HG-ESS) subtypes, distinguished by unique histopathological and molecular characteristics.
- LG-ESS progresses slowly and is associated with JAZF1-SUZ12 fusions, while HG-ESS is aggressive, marked by high mitotic activity, necrosis, and BCOR internal tandem duplication.
Purpose of the Study:
- To provide a comprehensive overview of the diagnosis, management, and prognosis of endometrial stromal sarcoma.
- To differentiate between the clinical and molecular features of LG-ESS and HG-ESS.
- To highlight current treatment strategies and emerging therapeutic avenues for ESS.
Main Methods:
- Review of histopathological and molecular features defining LG-ESS and HG-ESS.
- Analysis of surgical management, including hysterectomy and fertility-sparing options.
- Evaluation of adjuvant therapies, such as hormonal treatments and chemotherapy, based on ESS subtype.
Main Results:
- Surgical resection is the primary treatment for early-stage ESS; total hysterectomy with bilateral salpingo-oophorectomy is standard.
- LG-ESS shows favorable outcomes with hormonal therapy (aromatase inhibitors, progestins), while HG-ESS requires chemotherapy (e.g., doxorubicin-based regimens).
- Prognosis is stage and subtype-dependent; LG-ESS has >90% five-year survival in early stages, whereas HG-ESS has poorer survival rates.
Conclusions:
- ESS management requires subtype-specific approaches, with distinct treatments for LG-ESS and HG-ESS.
- Hormonal therapy is effective for LG-ESS, while chemotherapy is the mainstay for HG-ESS.
- Molecular profiling holds promise for developing personalized therapies to improve outcomes in rare ESS cases.
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