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Gynecologic Cancer InterGroup (GCIG) Consensus Review for Ovarian Sex Cord Stromal Tumors
  1. Isabelle Ray-Coquard, MD, PhD*,
  2. Jubilee Brown, MD,
  3. Philipp Harter, MD,
  4. Diane M. Provencher, MD§,
  5. Peter C. Fong, MD,
  6. Johanna Maenpaa, MD, PhD,
  7. Jonathan A. Ledermann, MD, FRCP#,
  8. Gunter Emons, MD, PhD**,
  9. Dominique Berton Rigaud, MD,,
  10. Rosalind M. Glasspool, PhD, MBBS, FRCP,,
  11. Delia Mezzanzanica, PhD§§ and
  12. Nicoletta Colombo, MD∥∥
  1. *Centre Léon Bérard, Lyon, France (GINECO);
  2. MD Anderson Cancer Center, Houston, TX (GOG);
  3. Kliniken-Essen-Mitte, Essen, Germany (AGO);
  4. §CHUM Hôpital Notre Dame, Montréal, Québec, Canada (NCIC-CTG);
  5. FMHS, University of Auckland, Auckland, New Zealand (ANZGOG);
  6. Tampere University Hospital, Tampere, Finland (NSGO);
  7. #UCL Cancer Institute, London, United Kingdom (NCRI MRC);
  8. **University Medecine, Göttingen, Germany (AGO);
  9. ††Institut de Cancérologie de l’Ouest René Gauducheau, Saint Herblain, France (GINECO);
  10. ‡‡Beatson West of Scotland Cancer Centre, Glasgow, United Kingdom (SGCTG);
  11. §§Fondazione IRCCS Instituto Nazionale dei Tumori, Milan, Italy (MITO); and
  12. ∥∥University of Milan–Bicocca, Milan, Italy (MaNGO)
  1. Address correspondence and reprint requests to Isabelle Ray-Coquard, MD, PhD, Centre Léon Bérard, 28, rue Laennec, 69373 Lyon Cedex 08, France. E-mail: Isabelle.ray-coquard{at}lyon.unicancer.fr

Abstract

Sex cord stromal tumors (SCST) are rare cancers of the ovarian area in adults. They constitute a heterogeneous group of tumors that develop from the sex cords and the ovarian stroma. These tumors are detected typically at an early stage, and they may recur as late as 30 years after the initial treatment. Because 70% of the patients present with stage I tumors, surgery represents the most important therapeutic arm. There are no data to support any kind of postoperative adjuvant treatment for patients with stage IA or IB SCSTs, given the indolent nature of these neoplasms and the overall good prognosis. The long natural history of the disease may lead to repeated surgical procedure should a relapse occurs. Platinum-based chemotherapy is currently used for patients with advanced stage SCSTs or recurrent disease, with an overall response rate of 63% to 80%. The indolent nature of SCSTs with the tendency for late recurrence requires long-term follow-up.

  • Rare tumor
  • Sex cord stromal tumors
  • Molecular analysis
  • First-line treatment
  • Metastatic disease

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Footnotes

  • The authors declare no conflicts of interest.

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