Skip to main content
Log in

High-grade endometrial stromal sarcoma versus undifferentiated uterine sarcoma: a Turkish uterine sarcoma group study-001

  • Gynecologic Oncology
  • Published:
Archives of Gynecology and Obstetrics Aims and scope Submit manuscript

Abstract

Objective

Prognostic factors associated with high-grade endometrial stromal sarcoma (HGESS) and undifferentiated uterine sarcoma (UUS) have not been distinctly determined due to the repetitive changes in the World Health Organization (WHO) classification. We aimed to compare clinicopathologic features and outcomes of patients with HGESS with those of patients with UUS.

Methods

A multi-institutional, retrospective, cohort study was conducted including 71 patients, who underwent surgery at 13 centers from 2008 to 2017. An experienced gynecopathologist from each institution re-evaluated the slides of their own cases according to the WHO2014 classification. Factors associated with refractory/progressive disease, recurrence or death were examined using logistic regression analyses. Kaplan–Meier method and log-rank test were used for survival comparisons.

Results

The median disease-free survival (DFS) for HGESS and UUS was 12 months and 6 months, respectively. While the median overall survival was not reached in HGESS group, it was 22 months in the UUS group. Kaplan–Meier analyses revealed that patients with UUS had a significantly poorer DFS than those with HGESS (p = 0.016), although OS did not differ between the groups (p = 0.135). Lymphovascular-space involvement (LVSI) was the sole significant factor associated with progression, recurrence or death for HGESS (Hazard ratio: 9.353, 95% confidence interval: 2.539–34.457, p = 0.001), whereas no significant independent factor was found for UUS.

Conclusions

UUS has a more aggressive behavior than HGESS. While no significant predictor of prognosis was found for UUS, LVSI is the sole independent prognostic factor for HGESS, with patients 9.3 times more likely to experience refractory/progressive disease, recurrence or death.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1

Similar content being viewed by others

References

  1. Hendrickson MR, Tavassoli F, Kempson RL, McCluggage G, Hailer U, Kubik-Huch RA (2003) Mesenchymal tumours and related lesions. In: Tavassoli FA, Deville P (eds) World Health Organization classification of tumours. Pathology and genetics of tumours of the breast and female organs. IARC Press, Lyon, France, pp 233–244

    Google Scholar 

  2. Kurman RJ, Carcangiu ML, Herrington CS, Young R (2014) WHO Classification of tumours of female reproductive organs. 4th Edition, Volume 6. IARC Press, Lyon, France: 135–50

  3. Parra-Herran C, Howitt BE (2019) Uterine mesenchymal tumors: update on classification, staging, and molecular features. Surg Pathol Clin 12(2):363–396. https://doi.org/10.1016/j.path.2019.01.004

    Article  PubMed  Google Scholar 

  4. NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines). Uterine Neoplasms, Version 1.2020. https://www.nccn.org. Accessed 29 May 2020

  5. Tanner EJ, Garg K, Leitao MM Jr, Soslow RA, Hensley ML (2012) High grade undifferentiated uterine sarcoma: surgery, treatment, and survival outcomes. Gynecol Oncol 127(1):27–31. https://doi.org/10.1016/j.ygyno.2012.06.030

    Article  PubMed  Google Scholar 

  6. Ríos I, Rovirosa A, Morales J, Gonzalez-Farre B, Arenas M, Ordi J, Pahisa Biete A (2014) Undifferentiated uterine sarcoma: a rare, not well known and aggressive disease: report of 13 cases. Arch Gynecol Obstet 290(5):993–997. https://doi.org/10.1007/s00404-014-3311-8

    Article  PubMed  Google Scholar 

  7. Malouf GG, Lhommé C, Duvillard P, Morice P, Haie-Meder C, Pautier P (2013) Prognostic factors and outcome of undifferentiated endometrial sarcoma treated by multimodal therapy. Int J Gynaecol Obstet 122(1):57–61. https://doi.org/10.1016/j.ijgo.2013.01.025

    Article  PubMed  Google Scholar 

  8. Schick U, Bolukbasi Y, Thariat J, Abdah-Bortnyak R, Kuten A, Igdem S, Caglar H, Ozsaran Z, Lössl K, Schleicher U, Zwahlen D, Villette S, Vees H (2012) Outcome and prognostic factors in endometrial stromal tumors: a Rare Cancer Network study. Int J Radiat Oncol Biol Phys 82(5):e757–e763. https://doi.org/10.1016/j.ijrobp.2011.11.005

    Article  PubMed  Google Scholar 

  9. Meurer M, Floquet A, Ray-Coquard I, Bertucci F, Auriche M, Cordoba A, Piperno-Neumann S, Salas S, Delannes M, Chevalier T, Italiano A, Blay JY, Mancini J, Pautier P, Duffaud F (2019) Localized high grade endometrial stromal sarcoma and localized undifferentiated uterine sarcoma: a retrospective series of the French Sarcoma Group. Int J Gynecol Cancer 29(4):691–698. https://doi.org/10.1136/ijgc-2018-000064

    Article  PubMed  Google Scholar 

  10. Prat J (2009) FIGO staging for uterine sarcomas. Int J Gynaecol Obstet 104(3):177–178. https://doi.org/10.1016/j.ijgo.2008.12.008

    Article  PubMed  Google Scholar 

  11. Philip CA, Pautier P, Duffaud F, Ray-Coquard I (2014) High-grade undifferentiated sarcomas of the uterus: diagnosis, outcomes, and new treatment approaches. Curr Oncol Rep 16(10):405. https://doi.org/10.1007/s11912-014-0405-1

    Article  CAS  PubMed  Google Scholar 

  12. Pautier P, Nam EJ, Provencher DM, Hamilton AL, Mangili G, Siddiqui NA, Westermann AM, Reed NS, Harter P, Ray-Coquard I (2014) Gynecologic Cancer InterGroup (GCIG) consensus review for high-grade undifferentiated sarcomas of the uterus. Int J Gynecol Cancer 24(9 Suppl 3):S73–S77. https://doi.org/10.1097/IGC.0000000000000281

    Article  PubMed  Google Scholar 

  13. Bartosch C, Exposito MI, Lopes JM (2010) Low-grade endometrial stromal sarcoma and undifferentiated endometrial sarcoma: a comparative analysis emphasizing the importance of distinguishing between these two groups. Int J Surg Pathol 18(4):286–291. https://doi.org/10.1177/1066896909337600

    Article  PubMed  Google Scholar 

  14. Baek MH, Park JY, Rhim CC, Kim JH, Park Y, Kim KR, Nam JH (2017) Investigation of new therapeutic targets in undifferentiated endometrial sarcoma. Gynecol Obstet Invest 82(4):329–339. https://doi.org/10.1159/000454769

    Article  CAS  PubMed  Google Scholar 

  15. Nordal RR, Thoresen SO (1997) Uterine sarcomas in Norway 1956–1992: incidence, survival and mortality. Eur J Cancer 33(6):907–911. https://doi.org/10.1016/s0959-8049(97)00040-3

    Article  CAS  PubMed  Google Scholar 

  16. Abeler VM, Nenodovic M (2011) Diagnostic immunohistochemistry in uterine sarcomas: a study of 397 cases. Int J Gynecol Pathol 30(3):236–243. https://doi.org/10.1097/PGP.0b013e318200caff

    Article  PubMed  Google Scholar 

  17. Ducimetière F, Lurkin A, Ranchère-Vince D, Decouvelaere AV, Péoc’h M, Istier L, Chalabreysse P, Muller C, Alberti L, Bringuier PP, Scoazec JY, Schott AM, Bergeron C, Cellier D, Blay JY, Ray-Coquard I (2011) Incidence of sarcoma histotypes and molecular subtypes in a prospective epidemiological study with central pathology review and molecular testing. PLoS ONE 6(8):e20294. https://doi.org/10.1371/journal.pone.0020294

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  18. Seagle BL, Shilpi A, Buchanan S, Goodman C, Shahabi S (2017) Low-grade and high-grade endometrial stromal sarcoma: a National Cancer Database study. Gynecol Oncol 146(2):254–262. https://doi.org/10.1016/j.ygyno.2017.05.036

    Article  PubMed  Google Scholar 

  19. Leath CA 3rd, Huh WK, Hyde J Jr, Cohn DE, Resnick KE, Taylor NP, Powell MA, Mutch DG, Bradley WH, Geller MA, Argenta PA, Gold MA (2007) A multi-institutional review of outcomes of endometrial stromal sarcoma. Gynecol Oncol 105(3):630–634. https://doi.org/10.1016/j.ygyno.2007.01.031

    Article  PubMed  Google Scholar 

  20. D’Angelo E, Prat J (2010) Uterine sarcomas: a review. Gynecol Oncol 116(1):131–139. https://doi.org/10.1016/j.ygyno.2009.09.023

    Article  CAS  PubMed  Google Scholar 

  21. Pautier P, Floquet A, Gladieff L, Bompas E, Ray-Coquard I, Piperno-Neumann S, Selle F, Guillemet C, Weber B, Largillier R, Bertucci F, Opinel P, Duffaud F, Reynaud-Bougnoux A, Delcambre C, Isambert N, Kerbrat P, Netter-Pinon G, Pinto N, Duvillard P, Haie-Meder C, Lhommé C, Rey A (2013) A randomized clinical trial of adjuvant chemotherapy with doxorubicin, ifosfamide, and cisplatin followed by radiotherapy versus radiotherapy alone in patients with localized uterine sarcomas (SARCGYN study). A study of the French Sarcoma Group. Ann Oncol 24(4):1099–1104

    Article  CAS  Google Scholar 

  22. Sciallis AP, Bedroske PP, Schoolmeester JK, Sukov WR, Keeney GL, Hodge JC, Bell DA (2014) High-grade endometrial stromal sarcomas: a clinicopathologic study of a group of tumors with heterogenous morphologic and genetic features. Am J Surg Pathol 38(9):1161–1172. https://doi.org/10.1097/PAS.0000000000000256

    Article  PubMed  Google Scholar 

  23. Hardell E, Josefson S, Ghaderi M, Skeie-Jensen T, Westbom-Fremer S, Cheek EH, Bell D, Selling J, Schoolmeester JK, Måsbäck A, Davidson B, Carlson JW (2017) Validation of a mitotic index cutoff as a prognostic marker in undifferentiated uterine sarcomas. Am J Surg Pathol 41(9):1231–1237. https://doi.org/10.1097/PAS.0000000000000894

    Article  PubMed  Google Scholar 

  24. Feng W, Malpica A, Robboy SJ, Gudlaugsson E, Hua K, Zhou X, Baak JP (2013) Prognostic value of the diagnostic criteria distinguishing endometrial stromal sarcoma, low grade from undifferentiated endometrial sarcoma, 2 entities within the invasive endometrial stromal neoplasia family. Int J Gynecol Pathol 32(3):299–306. https://doi.org/10.1097/PGP.0b013e318229adfb

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Contributions

AA: protocol/project development, supervision. MT: data collection or management. NB: data collection or management. GK: data collection or management. MG: data collection or management. TS: data collection or management. OO: data collection or management, manuscript editing. TT: data collection or management, data analysis, manuscript editing. IY: data collection or management, data analysis. MMM: protocol/project development, manuscript writing, supervision.

Corresponding author

Correspondence to Tayfun Toptas.

Ethics declarations

Conflict of interest

The authors declare that they have no conflicts of interest and nothing to disclose.

Ethical approval

This study has been approved by the Ethics Committee of the Saglik Bilimleri University, Ankara City Hospital (Date: 06/01/2020, Decision no.: 10). This article does not contain any studies with human participants or animals performed by any of the authors.

Informed consent

Informed consent was obtained from all patents.

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Ayhan, A., Tunc, M., Boran, N. et al. High-grade endometrial stromal sarcoma versus undifferentiated uterine sarcoma: a Turkish uterine sarcoma group study-001. Arch Gynecol Obstet 304, 475–483 (2021). https://doi.org/10.1007/s00404-020-05915-6

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00404-020-05915-6

Keywords

Navigation