Skip to main content

Advertisement

Log in

The Treatment Outcome for Adult Patients with Ewing’s Sarcoma

  • Sarcomas (SR Patel, Section Editor)
  • Published:
Current Oncology Reports Aims and scope Submit manuscript

Abstract

Ewing’s sarcoma is the second most common bone malignancy in children, but is extremely rare in adults. The outcome of patients with localized disease has improved over the past decades due to better combination chemotherapies, and better methods of local control. Unfortunately, patients with metastatic disease have a very poor outcome with current antineoplastic therapies. In this article, we will review the primary treatment for adult patients with Ewing’s sarcoma, both for localized and metastatic disease. The prognostic factors in adult patients with EWS will also be reviewed.

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.

Similar content being viewed by others

References

Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

  1. Ewing J. Diffuse endothelioma of bone. Proc of NY Path Soc. 1921;12:17.

    Google Scholar 

  2. Codman EA. Bone sarcomas: An interpretation of the nomenclature used by the committee on the registry of bone sarcomas of the American College of Surgeons. New York: Paul B Hoeber, Inc.; 1925.

    Google Scholar 

  3. Askin FB, Rosai J, Sibley RK, Dehner LP, McAlister WH. Malignant small cell tumor of the thoracopulmonary region in childhood A distinctive clininopathologic entity of uncertain histogenesis. Cancer. 1979;43:2438–51.

    Article  PubMed  CAS  Google Scholar 

  4. Turc-Carel C, Philip I, Berger MP, Philip T, Lenoir GM. Chromosome translocation in Ewing’s sarcoma. N Engl J Med. 1983;309:497–8.

    Google Scholar 

  5. Aurias A, Rimbaut C, Buffe D, Dubousset J, Mazabraud A. Chromosomal translocations Ewing’s sarcoma. N Engl J Med. 1983;309:496–7.

    Google Scholar 

  6. Delattre O, Zucman J, Plougastel B, Desmaze C, Melot T, Peter M, et al. Gene fusion with an ETS DNA-binding domain caused by chromosome translocation in human tumours. Nature. 1992;359(6391):162–5.

    Article  PubMed  CAS  Google Scholar 

  7. Zucman J, Delattre O, Desmaze C, Plougastel B, Joubert I, Melot T, et al. Cloning and characterization of the Ewing's sarcoma and peripheral neuroepithelioma t(11;22) translocation breakpoints. Genes Chromosomes Cancer. 1992;5(4):271–7.

    Article  PubMed  CAS  Google Scholar 

  8. Hromas R, Klemsz M. The ETS oncogene family in development, proliferation and neoplasia. Int J Hematol. 1994;59(4):257–65.

    PubMed  CAS  Google Scholar 

  9. Jawad MU, Cheung MC, Min ES, Schneiderbauer MM, Koniaris LG, Scully SP. Ewing’s sarcoma demonstrates racial disparities in incidence-related and sex-related differences in outcome. An analysis of 1631 cases from the SEER database, 1973–2005. Cancer. 2009;115:3526–36.

    Article  PubMed  Google Scholar 

  10. Cotterill SJ, Ahrens S, Paulussen M, Jurgens HF, Voute PA, Gadner H, et al. 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. 2000;18:3108–14.

    PubMed  CAS  Google Scholar 

  11. American Cancer Society. Facts & Figures 2013. http://www.cancer.org/research/cancerfactsfigures/indexer

  12. Bacci G, Ferrari S, Bertani F, Rimondini S, Longhi A, Bacchini P, et al. Prognostic factors in nonmetastatic Ewing’s sarcoma of bone treated with adjuvant chemotherapy: analysis of 359 patients at the Insttuto Ortopedicp Rizzoli. J Clin Oncol. 2000;18:4–11.

    PubMed  CAS  Google Scholar 

  13. Bacci G, Longhi A, Ferrari S, Mercuri M, Versari M, Bertoni F. Prognostic factors in non-metastatic Ewing’s sarcoma tumor of bone: an analysis of 579 patients treated at a single institution with adjuvant or neoadjuvant chemotherapy between 1972 and 1998. Acta Oncol. 2006;45:469–75.

    Article  PubMed  Google Scholar 

  14. Obata H, Ueda T, Kawai A, Ishii T, Ozaki T, Abe S, et al. Clinical outcome of patients with Ewing’s sarcoma family of tumors of bone in Japan: the Japanese Musculoskeletal Oncology Group Cooperative Study. Cancer. 2007;109(4):767–75.

    Article  PubMed  CAS  Google Scholar 

  15. Rosito P, Mancini AF, Rondelli R, Abate ME, Pession A, Bedei L, et al. Italian cooperative study for the treatment of children and young adults with localized Ewing’s sarcoma of bone: a preliminary report of 6 years of experience. Cancer. 1999;86(3):421–8.

    Article  PubMed  CAS  Google Scholar 

  16. Marina N, Grier H, Womer R, Granowetter L, Krailo M. Prognostic factors in Ewing’s sarcoma family of tumors (ES) treated in consecutive children’s oncology group (COG) studies: A report of 1444 patients. CTOS. 2010;40:886392.

    Google Scholar 

  17. Fizazi K, Dohollou N, Blay JY, Guerin S, Le Cesne A, Andre F, et al. anf Nguyes BB. Ewing’s family of tumors in adults: multivariate analysis of survival and long-term results of multimodality therapy in 182 patients. J Clin Oncol. 1998;16:3736–43.

    PubMed  CAS  Google Scholar 

  18. Landentein R, Potschger U, Le Deley MC, Whelan J, Paulussen M, Oberlin O, et al. primary disseminated multifocal Ewing’s sarcoma: results of Euro-Ewing 99 trial. J Clin Oncol. 2010;28:3284–91.

    Article  Google Scholar 

  19. Baldini EH, Demetri GD, Fletcher CDM, Foran J, Marcus KC, Singer S. Adults with Ewing’s sarcoma/Primitive neuroectodermal tumor. Adverse effect of older age and primary extraosseous disease on outcome. Ann Surg. 1999;230(1):79–86.

    Article  PubMed  CAS  Google Scholar 

  20. Martin RCG, Brennan MF. Adult soft tissue Ewing’s sarcoma or Primitive Neuroectodermal tumors. Arch Surg. 2003;138:281–5.

    Article  PubMed  Google Scholar 

  21. Verrill MW, Judson IR, Harmer CL, Fisher C, Thomas JM, Wiltshaw E. Ewing’s sarcoma and Primitive neuroectodermal tumor in adults: are they different from Ewing’s sarcoma and Primitive neuroectodermal tumor in children. J Clin Oncol. 1997;15:2611–21.

    PubMed  CAS  Google Scholar 

  22. Siegel RD, Ryan LM, Antman KH. Adults with Ewing’s sarcoma. An analysis of 16 patients at the Dana Farber Cancer Institute. Am J Clin Oncol. 1988;11(6):614–7.

    Article  PubMed  CAS  Google Scholar 

  23. Gupta AA, Pappo A, Saunders N, Hopyan S, Ferguson P, Wunder J, et al. Clinical outcome of children and adults with localized Ewing’s sarcoma. Cancer. 2010;116:3189–94.

    Article  PubMed  Google Scholar 

  24. Argon A, Basaran M, Yaman F, Dizdar Y, Sakar B, Camlica H, et al. Ewing’s sarcoma of the axial system in patients older than 15 years: dismal prognosis despite intensive multiagent chemotherapy and aggressive local treatment. Jpn J Clin Oncol. 2004;34(11):667–72.

    Article  PubMed  Google Scholar 

  25. Hense HW, Ahrens S, Paulussen M, Lehnert M, Jurgens H. Factors associated with tumor volume and primary metastases in Ewing’s tumors: Results from the (EI)CESS studies. Ann of Oncol. 1999;10:1073–7.

    Article  CAS  Google Scholar 

  26. Verril MW, Judson IR, Harmer CL, Fisher C, Thomas JM, Wiltshaw E. Ewing’s sarcoma and primitive neuroectodermal tumor in adults: arethey different from Ewing’s sarcoma and Primitive neuroectodermal tumor in children? J Clin Oncol. 1997;15:2611–21.

    Google Scholar 

  27. Bacci G, Ferrari S, Longhi A, Versari M, Forni C, Donati D, et al. Local and systemic control in Ewing’s sarcoma of the femur treated with chemotherapy, and locally by radiotherapy and/ or surgery. J Bone Joint Surg Br. 2003;85-B:107–14.

    Article  Google Scholar 

  28. Scully SP, Temple HT, O’Keefe RJ, Scarborough MT, Mankin HJ, Gebhardt MC. Role of surgical resection in pelvic Ewing’s sarcoma. J Clin Oncol. 1995;13:2336–41.

    PubMed  CAS  Google Scholar 

  29. Yock TI, Krailo M, Fryer CJ, Donaldson SS, Miser JS, Chen Z, et al. Local control in pelvic Ewing’s sarcoma: analysis from INT-0091- A report from the children’s oncology group. J Clin Oncol. 2006;24:3838–43.

    Article  PubMed  Google Scholar 

  30. •• Grier HE, Krailo MD, Tarbell NJ, Link MP, Fryer CJH, Pritchard DJ, et al. Addition of ifosfamide and etoposide to standard chemotherapy for Ewing’s sarcoma and Primitive neuroectodermal tumor of the bone. N Engl J Med. 2003;348(8):694–701. This randomized study with ifosfamide and etoposide added to standard therapy, shows an improvement in outcome in adult patients with localized disease. In addition, this study demonstrates that most adults can tolerate intensive chemotherapy regimens.

    Article  PubMed  CAS  Google Scholar 

  31. Juergens G, Weston C, Lewis I, Whelan J, Paulussen M, Oberlin O, et al. Safety Assessment of Intensive Induction With Vincristine, Ifosfamide, Doxorubicin, and Etoposide (VIDE) in the Treatment of Ewing Tumors in the EURO-E.W.I.N.G. 99 Clinical Trial. Pediatr Blood Cancer. 2006;47:22–9.

    Article  PubMed  Google Scholar 

  32. • Granowetter L, Womer R, Devidas M, Krailo M, Wang C, Bernstein M, et al. Dose-intensified compared with standard chemotherapy for nonmetastatic Ewing’s sarcoma family of tumors: a children’s oncology group study. J Clin Oncol. 2009;27:2536–41. This randomized study demonstrates that dose intensity, as given in this study, does not improve outcome in adult patients with Ewing’s sarcoma.

    Article  PubMed  CAS  Google Scholar 

  33. Sinkovics JG, Plager C, Ayala AG, Lindberg RD, Samuels ML. Ewing’s sarcoma: its course and treatment in 50 adult patients. Oncology. 1980;37:114–9.

    Article  PubMed  CAS  Google Scholar 

  34. Bacci G, Belladelli A, Forni C, Ferrari S, Longhi A, Bacchini P, et al. Adjuvant and neoadjuvant chemotherapy for Ewing’s sarcoma family tumors in patients aged between 40 and 60. Cancer. 2007;109:780–6.

    Article  PubMed  CAS  Google Scholar 

Download references

Conflict of Interest

Kristen N. Ganjoo declares no conflict of interest.

Shreyaskumar Patel has been a consultant for Novartis, Merck, GSK, and Johnson and Johnson and has received grant/research support from Infinity, Johnson and Johnson, PharmaMar, and Eisai.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Kristen N. Ganjoo.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Ganjoo, K.N., Patel, S. The Treatment Outcome for Adult Patients with Ewing’s Sarcoma. Curr Oncol Rep 15, 372–377 (2013). https://doi.org/10.1007/s11912-013-0317-5

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s11912-013-0317-5

Keywords

Navigation