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Gynecologic Cancer InterGroup (GCIG) Consensus Review for Endometrial Stromal Sarcoma
  1. Frédéric Amant, MD, PhD*,
  2. Anne Floquet, MD,
  3. Michael Friedlander, MBChB, FRACP, PhD,
  4. Gunnar Kristensen, MD, PhD§,
  5. Sven Mahner, MD, PhD,
  6. Eun Ji Nam, MD, PhD,
  7. Matthew A. Powell, MD#,
  8. Isabelle Ray-Coquard, MD, PhD**,
  9. Nadeem Siddiqui, FRCOG, PhD,,
  10. Peter Sykes, MBChB,,
  11. Anneke M. Westermann, MD, PhD§§ and
  12. Beatrice Seddon, MD, PhD∥∥
  1. *EORTC, Department of Oncology, KU Leuven and Department of Gynecologic Oncology, University Hospitals Leuven, Leuven, Belgium;
  2. GINECO, Department of Medical Oncology, Institut Bergonié, Bordeaux, France;
  3. ANZGOG, Department of Clinical Oncology, Prince of Wales Hospital, Randwick, Australia;
  4. §NSGO, Department for Gynecologic Oncology and Institute for Medical Informatics, Oslo University Hospital, Oslo, Norway;
  5. AGO, Department of Gynecology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany;
  6. KGOG, Women’s Cancer Clinic, Division of Gynecologic Oncology, Institute of Women’s Life Medical Science, Department of Obstetrics and Gynecology, Yonsei University College of Medicine, Seoul, South Korea;
  7. #GOG, Department of Obstetrics and Gynecology, Washington University School of Medicine, St. Louis, MO, United States of America,
  8. **GINECO, Centre Léon Bérard, Lyon, France;
  9. ††SGCTG, Department of Gynaecological Oncology, Glasgow Royal Infirmary, Glasgow, United Kingdom;
  10. ‡‡ANZGOG, Department of Obstetrics and Gynaecology, University of Otago, Christchurch, New Zealand;
  11. §§DGOG, Department of Medical Oncology, Academic Medical Center, Amsterdam, the Netherlands; and
  12. ∥∥MRCP, FRCR, Department of Oncology, University College Hospital London, London, United Kingdom.
  1. Address correspondence and reprint requests to Frédéric Amant, MD, PhD, Department of Oncology, KU Leuven, Gynecologic Oncology, University Hospitals Leuven, Herestraat 49, 3000 Leuven, Belgium. E-mail: frederic.amant{at}uzleuven.be.

Abstract

Abstract Endometrial stromal sarcoma (ESS) accounts for approximately 20% of all uterine sarcomas and presents, at a mean age, around 50 years of age. Half of the patients are premenopausal. ESS often manifests as an endometrial polyp and 60% of cases present with FIGO stage I disease. The natural history is one of slow growing indolent disease. Typical microscopic findings include a uniform population of endometrial stromal-type cells invading the myometrium and myometrial vessels. Imaging studies cannot reliably diagnose ESS preoperatively, so surgical resection for a presumed fibroid is a common scenario. Hysterectomy is the cornerstone of treatment for localized ESS, but morcellation should be avoided. Systematic lymphadenectomy in ESS does not improve the outcome. Leaving the ovaries in situ does not worsen survival and this is of importance especially for young women. The data support the current practice to administer adjuvant hormonal treatment, although several questions remain, such as optimal doses, regimens (progestins or aromatase inhibitors) and duration of therapy. Repeat surgery for recurrent disease that is indolent and hormone sensitive appears to be an acceptable approach. Systemic treatment for recurrent disease is mainly hormonal.

  • Surgical staging
  • Hormonal treatment
  • Surgery for relapse

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Footnotes

  • F.A. is a senior clinical researcher for the research fund Flanders (FWO - Fonds voor Wetenschappelijk Onderzoek).

  • The authors declare no conflicts of interest.

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