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Localized high grade endometrial stromal sarcoma and localized undifferentiated uterine sarcoma: a retrospective series of the French Sarcoma Group
  1. Marie Meurer1,2,
  2. A Floquet3,
  3. I Ray-Coquard4,
  4. F Bertucci5,
  5. M Auriche6,
  6. A Cordoba7,
  7. S Piperno-Neumann8,
  8. S Salas1,2,
  9. M Delannes9,
  10. T Chevalier1,2,
  11. A Italiano3,
  12. J Y Blay3,
  13. Julien Mancini10,11,
  14. P Pautier6 and
  15. F Duffaud1,2
  1. 1 Department of Medical Oncology, CHU Timone, AP-HM, Marseille, France
  2. 2 Aix-Marseille University, Marseille, France
  3. 3 Department of Medical Oncology, Institut Bergonié, Bordeaux, France
  4. 4 Department of Adult Medical Oncology, Centre Leon Berard and Claude Bernard University, Lyon, France
  5. 5 Department of Medical Oncology, Institut Paoli-Calmettes, Marseille, France
  6. 6 Department of Medicine, Gynecology Unit, Gustave Roussy, Villejuif, France
  7. 7 Department of General Cancerology, Oscar Lambret Center, Lille, France
  8. 8 Institut Curie, Paris, France
  9. 9 Department of Radiotherapy, Institut Claudius Regaud, Toulouse, France
  10. 10 Aix-Marseille Univ, INSERM, IRD, UMR 1252, SESSTIM, 'Cancers, Biomedicine and Society Group', Marseille, France
  11. 11 APHM, Timone Hospital, Public Health Department (BIOSTIC), Marseille, France
  1. Correspondence to Marie Meurer, Department of Medical Oncology, CHU Timone, AP-HM, Marseille 13005, France; meurermarie{at}


Objective High grade endometrial stromal sarcoma and undifferentiated uterine sarcomas are associated with a very poor prognosis. Although large surgical resection is the standard of care, the optimal adjuvant strategy remains unclear.

Methods A retrospective analysis of patients with localized high grade endometrial stromal sarcoma and undifferentiated uterine sarcomas (stages I–III) treated in 10 French Sarcoma Group centers was conducted.

Results 39 patients with localized high grade endometrial stromal sarcoma and undifferentiated uterine sarcomas treated from 2008 to 2016 were included. 24/39 patients (61.5%) were stage I at diagnosis. 38/39 patients underwent surgical resection, with total hysterectomy and bilateral oophorectomy completed in 26/38 (68%). Surgeries were mostly resection complete (R0, 23/38, 60%) and microscopically incomplete resection (R1, 6/38, 16%). 22 patients (58%) underwent postoperative radiotherapy (including brachytherapy in 11 cases), and 11 (29%) underwent adjuvant chemotherapy. After a median follow-up of 33 months (range 2.6–112), 17/39 patients were alive and 21/39 (54%) had relapsed (9 local relapses and 16 metastases). The 3 year and 5 year overall survival rates were 49.8% and 31.1%, respectively, and 3 year and 5 year disease free survival rates were 42.7% and 16.0%, respectively. Median overall survival and disease free survival were 32.7 (95% CI 16.3–49.1) and 23 (4.4–41.6) months, respectively. Medians were, respectively, 46.7 months and 39.0 months among those who underwent adjuvant radiotherapy and 41.0 months and 10.3 months for those who underwent adjuvant chemotherapy. In multivariate analysis, adjuvant radiotherapy was an independent prognostic factor for overall survival (P=0.012) and disease free survival (P=0.036). Chemotherapy, International Federation of Gynecology and Obstetrics I–II stages, and Eastern Cooperative Oncology Group-performance status 0 correlated with improved overall survival (P=0.034, P=0.002, P=0.006), and absence of vascular invasion (P=0.014) was associated with better disease free survival.

Conclusions The standard treatment of primary localized high grade endometrial stromal sarcoma and undifferentiated uterine sarcomas is total hysterectomy and bilateral oophorectomy. The current study shows that adjuvant radiotherapy and adjuvant chemotherapy appear to improve overall survival. A prospective large study is warranted to validate this therapeutic management.

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  • Contributors JM performed the statistical analysis. The other authors contributed to the recovery of patient data on the various French sites. FD supervised the study.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Patient consent Not required.

  • Ethics approval The registry was approved by the INCa (Institut National du Cancer) and local authorities (CNIL). RRePS and NETSARC databases are approved by the French Ethics Committee and National Agency CCTIRS (approval#09.594) and National Commission on Computing and Freedom (CNIL Approval # 90951018). The current study was a retrospective, minimal risk study, and all patients provided informed consented for use of their clinical data. Our institutional review board exempts such studies from requiring approval, and the FSG board of directors approved the study.

  • Provenance and peer review Not commissioned; externally peer reviewed.