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Prognostic factors and survival in Ewing’s sarcoma treated by limb salvage surgery

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A Correspondence to this article was published on 30 September 2019

Abstract

Purpose

Survival in Ewing’s sarcoma (ES) has increased with the use of chemotherapy. Surgical techniques such as limb salvage (LS) have been developed. Survival and adverse events have been widely studied in general series of ES, but there are few specific series of ES cases treated by LS, despite this being the most commonly used (surgical) approach. The aim of this study was to determine survival and prognostic factors in ES patients undergoing LS.

Patients and methods

We analysed all ES patients treated between January 1984 and May 2008 and selected all those treated by systemic multimodal therapy and LS. We assessed the influence of patient characteristics, tumour parameters and therapeutic results in event-free survival (EFS).

Results

Ninety patients were included. Fifty of them were treated by systemic multimodal therapy and locally by LS. ean age was 20 years. Overall survival (OS) was 68.8% and EFS was 60.6% at years. In the univariate analysis, pelvic location, age and response to chemotherapy were associated with poor prognosis. After multivariate analysis, poor response to treatment, pelvis location and age between 12 and 17 years were found to be independent prognostic factors. Dissemination at diagnosis was not a prognostic factor.

Conclusions

OS and EFS in ES treated by LS were similar to findings in previous ES studies. factors are no different, except for the presence of metastasis at diagnosis.

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References

  1. Burgert EO Jr, Nesbit ME, Garnsey LA, Gehan EA, Herrmann J, Vietti TJ, Cangir A, Tefft M, Evans R, Thomas P, et al. Multimodal therapy for the management of nonpelvic, localized Ewing's sarcoma of bone: intergroup study IESS-II. J Clin Oncol. 1990;8(9):1514–24.

    Article  PubMed  Google Scholar 

  2. Granowetter L, Womer R, Devidas M, Krailo M, Wang C, Bernstein M, Marina N, Leavey P, Gebhardt M, Healey J, Shamberger RC, Goorin A, Miser J, Meyer J, Arndt CAS, Sailer S, Marcus K, Perlman E, Dickman P, Grier HE. Dose-intensified compared with standard chemotherapy for nonmetastasic Ewing sarcoma family of tumors: a children’s oncology group study. J Clin Oncol. 2009;27(15):2536–41.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  3. Horowitz ME, Tsokos MG, DeLaney TF. Ewing’s sarcoma. CA Cancer J Clin. 1992;42(5):300–20.

    Article  CAS  PubMed  Google Scholar 

  4. Iwamoto Y. Diagnosis and treatment of Ewing’s sarcoma. Jpn J Clin Oncol. 2007;37(2):79–89.

    Article  PubMed  Google Scholar 

  5. Nesbit ME Jr, Gehan EA, Burgert EO Jr, Vietti TJ, Cangir A, Tefft M, Evans R, Thomas P, Askin FB, Kissane JM, et al. Multimodal therapy for the management of primary, nonmetastatic Ewing's sarcoma of bone: a long-term follow-up of the First Intergroup study. J Clin Oncol. 1990;8(10):1664–74.

    Article  PubMed  Google Scholar 

  6. Rosen G, Caparros B, Nirenberg A, Marcove RC, Huvos AG, Kosloff C, Lane J, Murphy ML. Ewing's sarcoma: ten-year experience with adjuvant chemotherapy. Cancer. 1981;47(9):2204–13.

    Article  CAS  PubMed  Google Scholar 

  7. Bacci G, Longhi A, Briccoli A, Bertoni F, Versari M, Picci P. The role of surgical margins in treatment of Ewing’s sarcomafamily tumors: experience of a single institution with 512 patients treated with adjuvant and neoadjuvant chemotherapy. Int J Radiat Oncol Biol Phys. 2006;65(3):766–72.

    Article  PubMed  Google Scholar 

  8. Krasin MJ, Davidoff AM, Rodriguez-Galindo C, Billups CA, Fuller CE, Neel MD, Merchant T. Definitive surgery and multiagent systemic therapy for patients with localized Ewing sarcoma family of tumors: local outcome and prognostic factors. Cancer. 2005;104(2):367–73.

    Article  PubMed  Google Scholar 

  9. Bacci G, Ferrari S, Bertoni F, Rimondini S, Longhi A, Bacchini P, Forni C, Manfrini M, Donati D, Picci P. Prognostic factors in nonmetastastic Ewing’s sarcoma of bone treated with adjuvant chemotherapy: analysis of 359 at the Instituto Ortopedico Rizzoli. J Clin Oncol. 2000;18(1):4–11.

    Article  CAS  PubMed  Google Scholar 

  10. Rodríguez-Galindo C, Liu T, Krasin MJ, Wu J, Billups CA, Daw NC, Sount SL, Rao BN, Santana VM, Navid F. Analysis of prognostic factors in Ewing sarcoma family of tumors. Cancer 2007; 112(2): 375–384.

  11. Aksnes LH, Bauer HC, Jebsen NL, Follerås G, Allert C, Haugen GS, Hall KS. Limb-sparing surgery preserves more function than amputation: a Scandinavian sarcoma group study of 118 patients. J Bone Joint Surg Br. 2008;29(6):786–94.

    Article  Google Scholar 

  12. Wunder JS, Paulian G, Huvos AG, Heller G, Meyers PA, Healey JH. The histological response to chemotherapy as a predictor of the oncological outcome of operative treatment of Ewing sarcoma. J Bone Joint Surg Am. 1998;80(7):1020–33.

    Article  CAS  PubMed  Google Scholar 

  13. Nesbit ME. Ewing’s sarcoma. CA Cancer J Clin. 1976;26(3):174–80.

    Article  CAS  PubMed  Google Scholar 

  14. Lin PP, Jaffe N, Herzog CE, Costelloe CM, Deavers MT, Kelly JS, Patel SR, Madewell JE, Lewis VO, Cannon CP, Benjamin RS, Yasko AW. Chemotherapy response is an important predictor of local recurrence in Ewing sarcoma. Cancer. 2007;109(3):603–11.

    Article  PubMed  Google Scholar 

  15. Sluga M, Windhager R, Lang S, Heinzl H, Kepler P, Mittermayer F, Dominkus M, Zoubek A, Kotz R. The role of surgery and resection margins in the treatment of Ewing’s sarcoma. Clin Orthop Relat Res. 2001;392:394–9.

    Article  Google Scholar 

  16. 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 

  17. Schuck A, Ahrens S, Paulussen M, Kuhlen M, Könemann S, Rübe C, Winkelmann W, Kotz R, Dunst J. Local therapy in localized Ewing tumors: results of 1058 patients treated in the CESS 81, CESS 86, and EICESS 92 trials. Int J Radiat Oncol Biol Phys. 2003;55(1):168–77.

    Article  PubMed  Google Scholar 

  18. San-Julian M, Dölz R, Garcia-Barrecheguren E, Noain E, Sierrasesumaga L, Cañadell J. Limb salvage in bone sarcomas in patients younger than age 10. A 20-year experience. J Pediatr Orthop 2003; 23(6):753–762.

  19. Bramer JA, Abudu AA, Grimer RJ, Carter SR, Tillman RM. Do pathological fractures influence survival and local recurrence rate in bony sarcomas? Eur J Cancer. 2007;43(13):1944–51.

    Article  CAS  PubMed  Google Scholar 

  20. Kepler P, Dominkus M, Toma CD, Kotz R. Endoprosthesis management of the extremities of children after resection of primary malignant bone tumors. Orthopade. 2003;32(11):1013–9.

    Article  Google Scholar 

  21. Cotterill SJ, Ahrens S, Paulussen M, Jürgens HF, Voûte PA, Gadner H, Craft AW. 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(17):3108–14.

    Article  CAS  PubMed  Google Scholar 

  22. Ladenstein R, Pötschger U, Le Deley MC, Whelan J, Paulussen, Oberlin O, van den Berg H, Dirksen U, Hjorth L, Michon J, Lewis I, Craft A, Jürgens H. Primary disseminated multifocal Ewing sarcoma: results of the Euro-Ewing 99 trial. J Clin Oncol 2010; 28(20):3284–3291.

  23. Picci P, Böhling T, Bacci G, Ferrari S, Sangiorgi L, Mercuri M, Ruggieri P, Manfrini M, Ferraro A, Casadei R, Benassi MS, Mancini AF, Rosito P, Cazzola A, Barbieri E, Tienghi A, del Prever BA, Comandone A, Bacchini P, Bertoni F. Chemotherapy-induced tumor necrosis as a prognostic factor in localized Ewing's sarcoma of the extremities. J Clin Oncol 1997; 15(4):1553-1559.

  24. Grier HE, Krailo MD, Tarbell NJ, Link MP, Fryer CJ, Pritchard DJ, Gebhardt MC, Dickman PS, Perlman EJ, Meyers PA, Donaldson SS, Moore S, Rausen AR, Vietti TJ, Miser JS. Addition of ifosfamide and etoposide to standard chemotherapy for Ewing's sarcoma and primitive neuroectodermal tumor of bone. N Engl J Med. 2003;348(8):694–701.

    Article  CAS  PubMed  Google Scholar 

  25. Dunst J, Sauer R, Burgers JM, Hawliczek R, Kürten R, Wiinkelmann W, Salzer-Kuntschik M, Müschenich M. Radiation therapy as local treatment in Ewing's sarcoma. Results of the Cooperative Ewing's Sarcoma Studies CESS 81 and CESS 86. Cancer 1991; 67(11): 2818-2825.

  26. Burnet NG, Bliss JM, Harmer CL. The impact of radiotherapy dose on local control of Ewing’s sarcoma of bone. Sarcoma. 1997;1:31–8.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  27. Paulussen M, Craft AW, Lewis I, Hackshaw A, Douglas C, Dunst J, Schuck A, Winkelmann W, Köhler G, Poremba C, Zoubek A, Ladenstein R, van den Berg H, Hunold A, Cassoni A, Spooner D, Grimer R, Whelan J, McTiernan A, Jürgens H. Results of the EICESS-92 study: two randomized trials of Ewing’s sarcoma treatment: cyclophosphamide compared with isfosfamide in standard-risk patients and assessment of benefit of etoposide added to standard treatment in high-risk patients. J Clin Oncol. 2008;26(27):4385–93.

    Article  CAS  PubMed  Google Scholar 

  28. Bacci G, Balladelli A, Forni C, Ferrari S, Longhi A, Bacchini P, Alberghini M, Fabbri N, Benassi MS, Briccoli A, Picci P. Adjuvant and neoadjuvant chemotherapy for Ewing sarcoma family of 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;19(4):780–6.

    Article  CAS  Google Scholar 

  29. Skubitz KM, D’Adamo DR. Sarcoma. Mayo Clin Proc. 2007;82(11):1409–32.

    Article  CAS  PubMed  Google Scholar 

  30. Desai SS, Jambhekar NA. Pathology of Ewing’s sarcoma/PNET: current opinion and emerging concepts. Indian J Orthop. 2010;44(4):363–8.

    Article  PubMed  PubMed Central  Google Scholar 

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Authors and Affiliations

  1. Department of Orthopaedic Surgery, Hospital Sant Joan Despí Moisès Broggi, C/ Jacint Verdaguer, 08970, Sant Joan Despí, Barcelona, Spain

    J. Alvarez-SanNicolas

  2. Department of Oncological Orthopaedic Surgery, Hospital de La Santa Creu I Sant Pau, Universitat Autònoma de Barcelona, Barcelona, Spain

    I. Gracia-Alegria, L. Trullols-Tarrago & A. Peiro-Ibañez

  3. Department of Orthopaedic Surgery, Hospital de La Santa Creu I Sant Pau, Universitat Autònoma de Barcelona, Barcelona, Spain

    C. Lamas-Gomez

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  1. J. Alvarez-SanNicolas
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Correspondence to J. Alvarez-SanNicolas.

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All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

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Alvarez-SanNicolas, J., Gracia-Alegria, I., Trullols-Tarrago, L. et al. Prognostic factors and survival in Ewing’s sarcoma treated by limb salvage surgery. Clin Transl Oncol 21, 1374–1382 (2019). https://doi.org/10.1007/s12094-019-02067-1

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