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Related Experiment Video

Updated: May 9, 2026

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Endometrial stromal sarcoma: a systematic review.

J Alejandro Rauh-Hain1, Marcela G del Carmen

  • 1Division of Gynecologic Oncology, Vincent Obstetrics and Gynecology, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts 02114, USA.

Obstetrics and Gynecology
|August 8, 2013
PubMed
Summary

Endometrial stromal sarcoma and undifferentiated endometrial sarcoma are rare uterine cancers. Surgical resection is key for early stages, while hormone therapy is effective for advanced or recurrent disease.

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Area of Science:

  • Gynecologic Oncology
  • Pathology
  • Reproductive Medicine

Background:

  • Endometrial stromal sarcoma (ESS) and undifferentiated endometrial sarcoma (UES) are rare uterine malignancies.
  • These tumors represent approximately 1% of all uterine cancers and less than 10% of uterine mesenchymal neoplasms.

Purpose of the Study:

  • To review and summarize existing studies on the evaluation and management of ESS and UES.
  • To provide insights into the epidemiology, pathogenesis, prognostic factors, and treatment strategies for these rare tumors.

Main Methods:

  • A systematic electronic literature search was performed using MEDLINE, PubMed, and ClinicalTrials.gov.
  • Included studies focused on ESS and UES, published in English between 1981 and 2013, with a review of 86 selected articles.

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  • Study quality was independently assessed, and data were systematically extracted.

    • Main Results:

    • Hysterectomy with bilateral salpingo-oophorectomy is the standard treatment for early-stage (I-II) ESS and UES.
    • Surgical resection is recommended for advanced-stage tumors when feasible.
    • Hormone therapy is the most effective treatment for advanced-stage or recurrent disease, while adjuvant therapy efficacy remains unproven for early stages.

    Conclusions:

    • ESS and UES are rare gynecologic tumors requiring specific management approaches.
    • Surgical intervention is crucial for both early and resectable advanced-stage disease.
    • Hormone therapy plays a significant role in managing advanced and recurrent cases.