Medical Oncology

, Volume 28, Supplement 1, pp 685–689

Treatment of adult patients with Ewing’s sarcoma: compliance with chemotherapy protocols & toxicity

Original Paper

Abstract

Data on treatment outcome of adult patients with Ewing’s sarcoma family of tumors (ESFT) are scarce. In some reports, adults with ESFT were found to have a worse outcome than children. The aim of this study was to assess compliance with chemotherapy protocols and toxicity and to compare two adult age groups. A retrospective analysis of clinical records of adult patients (≥21 years) treated at our center for ESFT from January 2000 to June 2010 was performed. Data recorded included demographics, clinical features, and outcomes. Patients were divided into two groups: ≥40 years (Group A) and <40 years (Group B). Twenty-two patients were included in the analysis, nine in Group A and 13 in Group B. Median age was 54 years in group A and 29 years in group B. Extra-osseous origin was diagnosed in 8/9 (89%) patients in group A and 8/13 (61%) patients in group B (P = 0.3). No significant differences between the two age groups regarding dose intensity, compliance with protocol, hematological and non-hematological toxicity, or overall survival were found. It was concluded that a multidisciplinary approach including multi-agent chemotherapy for a prolonged period of time is appropriate in adult patients with ESFT. Dose intensity of chemotherapy should be determined according to the patient’s clinical status and comorbidities. Treatment protocols used in the pediatric population, including high-dose chemotherapy, should be adapted for the adult population.

Keywords

Ewing’s sarcoma Peripheral primitive neuroectodermal tumor PNET Adults 

References

  1. 1.
    Delattre O, et al. The Ewing-family of tumors–a subgroup of small-round-cell tumors defined by specific chimeric transcripts. N Engl J Med. 1994;331:294–9.PubMedCrossRefGoogle Scholar
  2. 2.
    Bovée JV, Hogendoorn PC. Molecular pathology of sarcomas: concepts and clinical implications. Virchows Arch. 2010;456:193–9.PubMedCrossRefGoogle Scholar
  3. 3.
    Downing JR, et al. Detection of the (11;22)(q24;q12) translocation of Ewing’s sarcoma and peripheral neuroectodermal tumor by reverse transcription polymerase chain reaction. Am J Pathol. 1993;143:1294–300.PubMedGoogle Scholar
  4. 4.
    Rodriquez-Galindo C, Spunt SL, Pappo AS. Treatment of Ewing sarcoma family of tumors: current status and outlook for the future. Med Pediatr Oncol. 2003;40:276–87.CrossRefGoogle Scholar
  5. 5.
    Ladenstein R, et al. Primary disseminated multifocal Ewing sarcoma: results of the Euro-EWING 99 trial. J Clin Oncol. 2010;28:3284–91.PubMedCrossRefGoogle Scholar
  6. 6.
    Baldini EH, et al. Adults with Ewing’s sarcoma/primitive neuroectodermal tumor. Ann Surg. 1999;230:79–86.PubMedCrossRefGoogle Scholar
  7. 7.
    Rosito P, et al. Italian cooperative study for the treatment of children and young adults with localized Ewing sarcoma of bone: a preliminary report of 6 years of experience. Cancer. 1999;86:421–8.PubMedCrossRefGoogle Scholar
  8. 8.
    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;11:614–7.PubMedCrossRefGoogle Scholar
  9. 9.
    Fizazi K, et al. 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.PubMedGoogle Scholar
  10. 10.
    Bacci G, et al. Adjuvant and neoadjuvant chemotherapy for Ewing sarcoma family tumors in patients aged between 40 and 60: report of 35 cases and comparison of results with 586 younger patients treated with the same protocols in the same years. Cancer. 2007;109:780–6.PubMedCrossRefGoogle Scholar
  11. 11.
    Bacci G, et al. Neoadjuvant chemotherapy for Ewing’s sarcoma of bone in patients older than thirty-nine years. Acta Oncol. 2000;39:111–6.PubMedCrossRefGoogle Scholar
  12. 12.
    Maheshwari AV, Cheng EY. Ewing sarcoma family of tumors. J Am Acad Orthop Surg. 2010;18:94–107.PubMedGoogle Scholar
  13. 13.
    Wolden SL, Alektiar KM. Sarcomas across the age spectrum. Semin Radiat Oncol. 2010;20:45–51.PubMedCrossRefGoogle Scholar
  14. 14.
    Shi W, et al. Radiation treatment for Ewing family of tumors in adults: the University of Florida experience. Int J Radiat Oncol Biol Phys. 2008;72:1140–5.PubMedCrossRefGoogle Scholar
  15. 15.
    Verrill MW, et al. 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.PubMedGoogle Scholar
  16. 16.
    Lee J, Hoang BH, Ziogas A, Zell JA. Analysis of prognostic factors in Ewing sarcoma using a population-based cancer registry. Cancer. 2010;116:1964–73.PubMedCrossRefGoogle Scholar
  17. 17.
    El Weshi A, et al. Extraskeletal Ewing’s sarcoma family of tumours in adults: analysis of 57 patients from a single institution. Clin Oncol (R Coll Radiol). 2010;22:374–81.CrossRefGoogle Scholar
  18. 18.
    Bar-Sela G, Peer A, Rothschild S, Haim N. Treatment of patients aged over 50 years with non-osseous Ewing’s sarcoma family tumors: five cases and review of literature. Tumori. 2008;94:809–12.PubMedGoogle Scholar
  19. 19.
    Burgert EO, et al. Multimodal therapy for the management of nonpelvic, localized Ewing’s sarcoma of bone: intergroup study IESS II. J Clin Oncol. 1990;8:1514–24.PubMedGoogle Scholar
  20. 20.
    Grier HE, et al. Addition of ifosfamide and etoposide to standard chemotherapy for Ewing’s sarcoma and primitive neuroectodermal tumor of bone. N Engl J Med. 2003;348:694–701.PubMedCrossRefGoogle Scholar
  21. 21.
    Sinkovics JG, Plager C, Ayala AG, Lindberg RD, Samuels ML. Ewing sarcoma: its course and treatment in 50 adult patients. Oncology. 1980;37:114–9.PubMedCrossRefGoogle Scholar
  22. 22.
    Kinsella TJ, et al. Long-term follow-up of Ewing’s sarcoma of bone treated with combined modality therapy. Int J Radiat Oncol Biol Phys. 1991;20:389–95.PubMedCrossRefGoogle Scholar
  23. 23.
    Picci P, et al. Chemotherapy-induced tumor necrosis as a prognostic factor in localized Ewing’s sarcoma of the extremities. J Clin Oncol. 1997;15:1553–9.PubMedGoogle Scholar
  24. 24.
    Martin RC 2nd, Brennan MF. Adult soft tissue Ewing sarcoma or primitive neuroectodermal tumors: predictors of survival? Arch Surg. 2003;138:281–5.PubMedCrossRefGoogle Scholar
  25. 25.
    Smorenburg CH, van Groeningen CJ, Meijer OW, Visser M, Boven E. Ewing’s sarcoma and primitive neuroectodermal tumour in adults: single-centre experience in The Netherlands. Neth J Med. 2007;65:132–6.PubMedGoogle Scholar
  26. 26.
    Paulussen M, et al. Localized Ewing tumor of bone: final results of the cooperative Ewing’s sarcoma study CESS 86. J Clin Oncol. 2001;19:1818–29.PubMedGoogle Scholar
  27. 27.
    Paulussen M, et al. European intergroup cooperative Ewing’s sarcoma study-92. Results of the EICESS-92 study: two randomized trials of Ewing’s sarcoma treatment–cyclophosphamide compared with ifosfamide in standard-risk patients and assessment of benefit of etoposide added to standard treatment in high-risk patients. J Clin Oncol. 2008;26:4385–93.PubMedCrossRefGoogle Scholar
  28. 28.
    Castex MP, et al. Extraosseous localized ewing tumors: improved outcome with anthracyclines—the French society of pediatric oncology and international society of pediatric oncology. J Clin Oncol. 2007;25:1176–82.PubMedCrossRefGoogle Scholar
  29. 29.
    El Weshi A, et al. VIP (etoposide, ifosfamide, cisplatin) in adult patients with recurrent or refractory Ewing sarcoma family of tumors. Am J Clin Oncol. 2004;27:529–34.PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2010

Authors and Affiliations

  1. 1.Division of Oncology, Rambam-Health Care Campus and Faculty of MedicineTechnion-Israel Institute of TechnologyHaifaIsrael

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