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Ewing Sarcoma

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Bone Sarcoma

Part of the book series: MD Anderson Cancer Care Series ((MDCCS))

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Ewing sarcoma is a disease characterized by small, undifferentiated cells. Although their exact histogenesis is not known, it is believed that they may derive from mesenchymal stem cells in bone marrow. The age distribution at diagnosis shows a sharp peak during the second decade, with rare cases in the elderly. Chemotherapy is an essential part of therapy. Vincristine, doxorubicin, cyclophosphamide, dactinomycin, ifosfamide, and etoposide are considered active agents. A positive response to preoperative therapy can be manifested by marked shrinkage of the extraosseous tumor or ossification of the tumor. Local therapy for the primary tumor is usually achieved by wide surgical excision. Radiation is employed without surgery for tumors in certain difficult locations, such as the spine and cranium. Surgery and radiation are occasionally used together, but the risk of complications with the combined treatment is increased. For patients who present with localized disease, the 5-year overall survival rate is approximately 60–70%. The prognosis is markedly worse for patients with metastatic disease.

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Suggested Readings

  • Burgert Jr EO, Nesbit ME, Garnsey LA, 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.

    PubMed  Google Scholar 

  • Cotterill SJ, Ahrens S, Paulussen M, 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 

  • Craft A, Cotterill S, Malcolm A, et al. Ifosfamide-containing chemotherapy in Ewing’s sarcoma: the Second United Kingdom Children’s Cancer Study Group and the Medical Research Council Ewing’s Tumor Study. J Clin Oncol. 1998;16:3628–33.

    PubMed  CAS  Google Scholar 

  • Donaldson SS, Torrey M, Link MP, et al. A multidisciplinary study investigating radiotherapy in Ewing’s sarcoma: end results of POG #8346. Pediatric Oncology Group. Int J Radiat Oncol Biol Phys. 1998;42:125–35.

    Article  PubMed  CAS  Google Scholar 

  • Evans RG, Nesbit ME, Gehan EA, et al. Multimodal therapy for the management of localized Ewing’s sarcoma of pelvic and sacral bones: a report from the second intergroup study. J Clin Oncol. 1991;9:1173–80.

    PubMed  CAS  Google Scholar 

  • Grier HE, Krailo MD, Tarbell NJ, 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.

    Article  PubMed  CAS  Google Scholar 

  • Kushner BH, Meyers PA. How effective is dose-intensive/myeloablative therapy against Ewing’s sarcoma/primitive neuroectodermal tumor metastatic to bone or bone marrow? The Memorial Sloan-Kettering experience and a literature review. J Clin Oncol. 2001;19:870–80.

    PubMed  CAS  Google Scholar 

  • Lin PP, Jaffe N, Herzog CE, et al. Chemotherapy response is an important predictor of local recurrence in Ewing sarcoma. Cancer. 2007;109:603–11.

    Article  PubMed  Google Scholar 

  • Mackintosh C, Madoz-Gurpide J, Ordonez JL, et al. The molecular pathogenesis of Ewing’s sarcoma. Cancer Biol Ther. 2010;9:655–67.

    Article  PubMed  CAS  Google Scholar 

  • Nesbit Jr ME, Gehan EA, Burgert Jr EO, 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;8:1664–74.

    PubMed  Google Scholar 

  • Olmos D, Postel-Vinay S, Molife LR, et al. Safety, pharmacokinetics, and preliminary activity of the anti-IGF-1R antibody figitumumab (CP-751,871) in patients with sarcoma and Ewing’s sarcoma: a phase 1 expansion cohort study. Lancet Oncol. 2010;11:129–35.

    Article  PubMed  CAS  Google Scholar 

  • Paulussen M, Ahrens S, Craft AW, et al. Ewing’s tumors with primary lung metastases: survival analysis of 114 (European Intergroup) Cooperative Ewing’s Sarcoma Studies patients. J Clin Oncol. 1998;16:3044–52.

    PubMed  CAS  Google Scholar 

  • Paulussen M, Ahrens S, Dunst J, 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.

    PubMed  CAS  Google Scholar 

  • Rosito P, Mancini AF, Rondelli R, 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 [Note: dosage error in text. Erratum appears in Cancer 2005;104:667.].

    Article  PubMed  CAS  Google Scholar 

  • Subbiah V, Naing A, Brown RE, et al. Targeted morphoproteomic profiling of Ewing’s sarcoma treated with insulin-like growth factor 1 receptor (IGF1R) inhibitors: response/resistance signatures. PLoS One. 2011;6:e18424.

    Article  PubMed  CAS  Google Scholar 

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Authors and Affiliations

  1. Department of Orthopaedic Oncology, Unit 1448, Division of Surgery, The University of Texas MD Anderson Cancer Center, 301402, Houston, TX, 77230, USA

    Patrick P. Lin

  2. Department of Pediatrics, Unit 87, Division of Pediatrics, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, TX, 77030, USA

    Cynthia E. Herzog

  3. Department of Radiation Oncology, Unit 97, Division of Radiation Oncology, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, TX, 77030, USA

    Ashleigh Guadagnolo

  4. Department of Sarcoma Medical Oncology, Unit 450, Division of Cancer Medicine, The University of Texas MD Anderson Cancer Center, 1400 Holcombe Boulevard, Houston, TX, 77030, USA

    Shreyaskumar Patel

Authors
  1. Patrick P. Lin
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  2. Cynthia E. Herzog
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  3. Ashleigh Guadagnolo
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  4. Shreyaskumar Patel
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Corresponding author

Correspondence to Patrick P. Lin .

Editor information

Editors and Affiliations

  1. Division of Surgery, Department of Orthopedic Oncology, The University of Texas M.D. Anderson Cancer Center, Houston, 77030, Texas, USA

    Patrick P. Lin

  2. Division of Cancer Medicine, Department of Sarcoma Medical Oncology, The University of Texas M.D. Anderson Cancer Center, Houston, 77030, Texas, USA

    Shreyaskumar Patel

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Lin, P.P., Herzog, C.E., Guadagnolo, A., Patel, S. (2013). Ewing Sarcoma. In: Lin, P., Patel, S. (eds) Bone Sarcoma. MD Anderson Cancer Care Series. Springer, Boston, MA. https://doi.org/10.1007/978-1-4614-5194-5_6

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  • DOI: https://doi.org/10.1007/978-1-4614-5194-5_6

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  • Publisher Name: Springer, Boston, MA

  • Print ISBN: 978-1-4614-5193-8

  • Online ISBN: 978-1-4614-5194-5

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