Skip to main content

Advertisement

SpringerLink
Log in

Prognostic factors for overall survival in paediatric patients with Ewing sarcoma of bone treated according to multidisciplinary protocol

  • Research Articles
  • Published:
Clinical and Translational Oncology Aims and scope Submit manuscript

Abstract

Background

The purpose of this study is to assess the outcome of patients with Ewing sarcoma (EWS) of the bone and to identify prognostic factors.

Materials and methods

Seventy-seven patients younger than 18 years old, diagnosed with EWS of the bone between 1979 and 2009, were analysed retrospectively. Four different protocols of chemotherapy were used successively. Local treatment consisted of surgery (N=32), radiotherapy (N=20) and a combination of both (N=19).

Results

The median age at diagnosis was 10 years old (range, 2–17) and the median follow-up for survivors 8.6 years (range, 1–18.8). Thirty-two relapses occurred (21 distant, 5 local and 6 both). The 2- and 5-year overall survival rates were 70% and 51%, respectively. Multivariate analysis showed four significant independent predictors for death: age ≥14 years old (HR: 5.06; p=0.019), lack of complete response (HR: 8.04; p<0.001), tumour volume ≥150 ml (HR: 2.21; p=0.045) and distant recurrences (HR: 1.45; p=0.001).

Conclusions

Outcome of EWS of bone is influenced by many clinical and treatment-correlated variables. Criteria to stratify patients should include all the variables that have shown prognostic significance. The development of novel therapies should target these high-risk groups.

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

Access this article

Log in via an institution

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

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  1. de Alava E (2007) Molecular pathology in sarcomas. Clin Transl Oncol 9:130–144

    Article  PubMed  Google Scholar 

  2. Rodriguez-Galindo C, Liu T, Krasin MJ et al (2007) Analysis of prognostic factors in ewing sarcoma family of tumors: review of St. Jude Children’s Research Hospital studies. Cancer 110:375–384

    Article  PubMed  Google Scholar 

  3. Bernstein M, Kovar H, Paulussen M et al (2005) Ewing sarcoma family of tumors: Ewing sarcoma of bone and soft tissue and the peripheral primitive neuroectodermal tumors. In: Pizzo P, Poplack D (eds) Principles and practice of pediatric oncology, 5th edn. Lippincott Williams and Wilkins, Philadelphia, pp 1002–1032

    Google Scholar 

  4. Peris-Bonet R (2008) Incidencia y supervivencia del cancer infantil. Rev Esp Pediatr 64:342–356

    Google Scholar 

  5. Craft AW, Paulussen M, Douglas C et al (2000) EICESS 92: early results of an international Ewings tumour study. Med Pediatr Oncol 35:191

    Google Scholar 

  6. Grier HE, Krailo MD, Tarbell NJ et al (2003) Addition of ifosfamide and etoposide to standard chemotherapy for Ewing’s sarcoma and primitive neuroectodermal tumor of bone. N Engl J Med 348:694–701

    Article  PubMed  CAS  Google Scholar 

  7. Rodriguez-Galindo C, Spunt SL, Pappo AS (2003) Treatment of Ewing sarcoma family of tumors: current status and outlook for the future. Med Pediatr Oncol 40:276–287

    Article  PubMed  Google Scholar 

  8. Cotterill SJ, Ahrens S, Paulussen M et al (2000) Prognostic factors in Ewing’s tumor of bone: analysis of 975 patients from the European Intergroup Cooperative Ewing’s Sarcoma Study Group. J Clin Oncol 18:3108–3114

    PubMed  CAS  Google Scholar 

  9. Miser JS, Goldsby RE, Chen Z et al (2007) Treatment of metastatic Ewing sarcoma/primitive neuroectodermal tumor of bone: evaluation of increasing the dose intensity of chemotherapy-a report from the Children’s Oncology Group. Pediatr Blood Cancer 49:894–900

    Article  PubMed  Google Scholar 

  10. American Joint Committee on Cancer (2002) Bone. In: Greene FL, Page DL, Fleming ID et al (eds) AJCC cancer staging manual. Springer-Verlag, New York, NY, pp 213–219

    Google Scholar 

  11. Rosen G, Caparros B, Nirenberg A et al (1981) Ewing’s sarcoma: ten-year experience with adjuvant chemotherapy. Cancer 47:2204–2213

    Article  PubMed  CAS  Google Scholar 

  12. Kushner BH, Meyers PA, Gerald WL et al (1995) Very-high-dose short-term chemotherapy for poorrisk peripheral primitive neuroectodermal tumors, including Ewing’s sarcoma, in children and young adults. J Clin Oncol 13:2796–2804

    PubMed  CAS  Google Scholar 

  13. Donaldson SS, Torrey M, Link MP et al (1998) A multidisciplinary study investigating radiotherapy in Ewing’s sarcoma: end results of POG #8346. Pediatric Oncology Group. Int J Radiat Oncol Biol Phys 42:125–135

    Article  PubMed  CAS  Google Scholar 

  14. WHO (1979) Handbook for reporting results of cancer treatment. World Health Organization, Geneva, Offset Publication No. 48

  15. Therasse P, Arbuck SG, Eisenhauer EA et al (2000) New guidelines to evaluate the response to treatment in solid tumors. European Organization for Research and Treatment of Cancer, National Cancer Institute of the United States, National Cancer Institute of Canada. J Natl Cancer Inst 92:205–216

    Article  PubMed  CAS  Google Scholar 

  16. Kaplan EL, Meier P (1958) Nonparametric estimation from incomplete observations. J Am Stat Assoc 53:457–481

    Google Scholar 

  17. Bacci G, Ferrari S, Mercuri M et al (2003) Multimodal therapy for the treatment of nonmetastatic Ewing sarcoma of pelvis. J Pediatr Hematol Oncol 25:118–124

    Article  PubMed  Google Scholar 

  18. Rodriguez-Galindo C, Navid F, Liu T et al (2008) Prognostic factors for local and distant control in Ewing sarcoma family of tumors. Ann Oncol 19:814–820

    Article  PubMed  CAS  Google Scholar 

  19. Peng RJ, Sun XF, Xiang XJ et al (2009) Efficacy and survival of 92 cases of Ewing’s sarcoma family of tumor initially treated with multidisciplinary therapy. Ai Zheng 28:1304–1309

    PubMed  Google Scholar 

  20. Yonemori K, Yamaguchi U, Kaneko M et al (2008) Prediction of response and prognostic factors for Ewing family of tumors in a low incidence population. J Cancer Res Clin Oncol 34:389–395

    Article  Google Scholar 

  21. Jurgens H, Exner U, Gadner H et al (1988) Multidisciplinary treatment of primary Ewing’s sarcoma of bone. A 6-year experience of a European Cooperative Trial. Cancer 61:23–32

    Article  PubMed  CAS  Google Scholar 

  22. Paulussen M, Ahrens S, Dunst J et al (2001) Localized Ewing tumor of bone: final results of the cooperative Ewing’s Sarcoma Study CESS 86. J Clin Oncol 19:1818–1829

    PubMed  CAS  Google Scholar 

  23. Bacci G, Ferrari S, Longhi A et al (2003) Therapy and survival after recurrence of Ewing’s tumors: the Rizzoli experience in 195 patients treated with adjuvant and neoadjuvant chemotherapy from 1979 to 1997. Ann Oncol 14:1654–1659

    Article  PubMed  CAS  Google Scholar 

  24. Rodriguez-Galindo C, Billups CA, Kun LE et al (2002) Survival after recurrence of Ewing tumors: the St Jude Children’s Research Hospital experience, 1979–1999. Cancer 94:561–569

    Article  PubMed  Google Scholar 

  25. Bacci G, Forni C, Longhi A et al (2004) Longterm outcome for patients with non-metastatic Ewing’s sarcoma treated with adjuvant and neoadjuvant chemotherapies. 402 patients treated at Rizzoli between 1972 and 1992. Eur J Cancer 40:73–83

    Article  PubMed  CAS  Google Scholar 

  26. Lee J, Hoang BH, Ziogas A, Zell JA (2010) Analysis of prognostic factors in Ewing sarcoma using a population-based cancer registry. Cancer 116:1964–1973

    Article  PubMed  Google Scholar 

  27. Shafat I, Ben-Arush MW, Issakov J et al (2010) Preclinical and clinical significance of heparanase in Ewing’s sarcoma. J Cell Mol Med 15:1857–1864

    Article  Google Scholar 

  28. Sari N, Togral G, Cetindag MF et al (2010) Treatment results of the Ewing sarcoma of bone and prognostic factors. Pediatr Blood Cancer 54:19–24

    Article  PubMed  Google Scholar 

  29. Leavey PJ, Mascarenhas L, Marina N et al (2008) Prognostic factors for patients with Ewing sarcoma (EWS) at first recurrence following multimodality therapy: a report from the Children’s Oncology Group. Pediatr Blood Cancer 51:334–338

    Article  PubMed  Google Scholar 

  30. Miser JS, Kinsella TJ, Triche TJ et al (1987) Ifosfamide with mesna uroprotection and etoposide: an effective regimen in the treatment of recurrent sarcomas and other tumors of children and young adults. J Clin Oncol 5:1191–1198

    PubMed  CAS  Google Scholar 

  31. Jurgens H, Exner U, Kuhl J et al (1989) High-dose ifosfamide with mesna uroprotection in Ewing’s sarcoma. Cancer Chemother Pharmacol 24[Suppl 1]:S40–44

    PubMed  Google Scholar 

  32. Pinkerton CR, Rogers H, James C et al (1985) A phase II study of ifosfamide in children with recurrent solid tumours. Cancer Chemother Pharmacol 15:258–262

    Article  PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Radiation Oncology, The University of Texas M.D. Anderson Cancer Center, 1515 Holcombe Blvd, Unit 97, Houston, TX, 77030, USA

    José Luis López Guerra

  2. Department of Radiation Oncology, Virgen del Rocío University Hospital, Sevilla, Spain

    José Luis López Guerra, Patricia Cabrera, Rafael Ordóñez & María José Ortiz

  3. Department of Pediatric Oncology, Virgen del Rocío University Hospital, Sevilla, Spain

    Catalina Márquez-Vega & Gema Lucía Ramírez-Villar

  4. Methodology Unit Fundación Pública Andaluza para la Gestión de la Investigación en Salud de Sevilla, Virgen del Rocío University Hospital, Sevilla, Spain

    Juan Manuel Praena-Fernández

Authors
  1. José Luis López Guerra
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Catalina Márquez-Vega
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Gema Lucía Ramírez-Villar
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Patricia Cabrera
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Rafael Ordóñez
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Juan Manuel Praena-Fernández
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. María José Ortiz
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to José Luis López Guerra.

Rights and permissions

Reprints and permissions

About this article

Cite this article

López Guerra, J.L., Márquez-Vega, C., Ramírez-Villar, G.L. et al. Prognostic factors for overall survival in paediatric patients with Ewing sarcoma of bone treated according to multidisciplinary protocol. Clin Transl Oncol 14, 294–301 (2012). https://doi.org/10.1007/s12094-012-0798-y

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s12094-012-0798-y

Keywords

Search

Navigation