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Ewing’s sarcoma

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Opinion statement

Patients with Ewing’s sarcoma should be transferred to a comprehensive cancer center for evaluation and management when the diagnosis is suspected. Proper biopsy technique is essential to preserve all therapeutic options, including limb preservation surgery. In addition to conventional histologic examination, biopsy tissue must be obtained for molecular biology studies. Demonstration of the consistent chromosomal translocation associated with Ewing’s sarcoma is essential for diagnosis, and the specific type of fusion transcript has prognostic implications. Treatment must be intimately coordinated among oncologist, surgeon, and radiation oncologist. Successful treatment requires systemic, multi-agent chemotherapy and local control. The primary tumor can be treated with surgery, radiation therapy, or a combination of the two. The choice of modality should be dictated by the age of the patient, location of the primary tumor, functional consequences of the intervention, and concern about late effects, especially secondary malignancy. Treatment of the patient who presents with clinically detectable metastatic disease or who relapses after initial therapy remains unsatisfactory and controversial.

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References and Recommended Reading

  1. Delattre O, Zucman J, Melot T, et al.: The Ewing family of tumors—a subgroup of small-round-cell tumors defined by specific chimeric transcripts. N Engl J Med 1994, 331:294–299. This study covers delineation of the unique chromosomal translocation and specific fusion transcripts associated with Ewing’s sarcoma. The identification of these transcripts led to improved precision in diagnosis, greater sensitivity for detection of minimal or residual disease, and key clues to the pathogenesis of Ewing’s sarcoma.

    Article  PubMed  CAS  Google Scholar 

  2. Horowitz ME, Malawar MM, Soo SY, Hicks MJ: Ewing’s sarcoma family of tumors: Ewing’s sarcoma of bone and soft tissue and the peripheral primitive neuroectodermal tumors. In Principles and Practice of Pediatric Oncology, edn 3. Edited by Pizzo and Poplack. Philadelphia: Lippincott Williams & Wilkins; 1997:844–845.

    Google Scholar 

  3. Desmaze C, Brizard F, Turc-Carel C, et al.: Multiple chromosomal mechanisms generate an EWS/FLI1 or an EWS/ERG fusion gene in Ewing tumors. Cancer Genet Cytogenet 1997, 97:12–19.

    Article  PubMed  CAS  Google Scholar 

  4. Desmaze C, Zucman J, Delattre O, et al.: Unicolor and bicolor in situ hybridization in the diagnosis of peripheral neuroepithelioma and related tumors. Genes Chromosomes Cancer 1992, 5:30–34.

    Article  PubMed  CAS  Google Scholar 

  5. Ladanyi M, Lewis R, Garin-Chesa P, et al.: EWS rearrangement in Ewing’s sarcoma and peripheral neuroectodermal tumor. Molecular detection and correlation with cytogenetic analysis and MIC2 expression. Diagn Mol Pathol 1993, 2:141–146.

    PubMed  CAS  Google Scholar 

  6. Grier H, Krailo M, Link M, et al.: Improved outcome in nonmetastatic Ewing’s sarcoma (EWS) and PNET of bone with the addition of ifosfamide (I) and etoposide (E) to vincristine (V), Adriamycin (Ad), cyclophosphamide (C), and actinomycin (A): a Children’s Cancer Group (CCG) and Pediatric Oncology Group (POG) report. Proc Am Soc Clin Oncol 1994, 13:A1443. The addition of ifosfamide and etoposide to cyclophosphamide, doxorubicin, and vincristine significantly improves the probability for event-free survival in Ewing’s sarcoma.

    Google Scholar 

  7. Miser JS, Krailo M, Meyers P, et al.: Metastatic Ewing’s sarcoma (ES) and primitive neuroectodermal tumor (PNET) of bone; failure of new regimens to improve outcome. Proc Am Soc Clin Oncol 1996, 15:A1472.

    Google Scholar 

  8. Oberlin O, Habrand JL, Zucker JM, et al.: No benefit of ifosfamide in Ewing’s sarcoma: a nonrandomized study of the French Society of Pediatric Oncology. J Clin Oncol 1992, 10:1407–1412.

    PubMed  CAS  Google Scholar 

  9. Kushner BH, Meyers PA, Gerald WL, et al.: Very-highdose short-term chemotherapy for poor-risk peripheral primitive neuroectodermal tumors, including Ewing’s sarcoma, in children and young adults. J Clin Oncol 1995, 13:2796–2804.

    PubMed  CAS  Google Scholar 

  10. Thomas PR, Perez CA, Neff JR, et al.: The management of Ewing’s sarcoma: role of radiotherapy in local tumor control. Cancer Treat Rep 1984, 68:703–710.

    PubMed  CAS  Google Scholar 

  11. Dunst J, Sauer R, Burgers JM, et al.: Radiation therapy as local treatment in Ewing’s sarcoma. Results of the Cooperative Ewing’s Sarcoma Studies CESS 81 and CESS 86. Cancer 1991, 67:2818–2825.

    Article  PubMed  CAS  Google Scholar 

  12. Merchant TE, Kushner BH, Sheldon JM, et al.: Effect of low-dose radiation therapy when combined with surgical resection for Ewing sarcoma. Med Pediatr Oncol 1999, 33:65–70.

    Article  PubMed  CAS  Google Scholar 

  13. Kuttesch JF Jr, Wexler LH, Marcus RB, et al.: Second malignancies after Ewing’s sarcoma: radiation dose-dependency of secondary sarcomas. J Clin Oncol 1996, 14:2818–2825.

    PubMed  Google Scholar 

  14. Wunder JS, Paulian G, Huvos AG, et al.: The histological response to chemotherapy as a predictor of the oncological outcome of operative treatment of Ewing sarcoma. J Bone Joint Surg Am 1998, 80:1020–1033. Patients with Ewing’s sarcoma typically receive induction chemotherapy. When they undergo surgery for definitive local control, the degree of necrosis in the primary tumor correlates with the subsequent probability for event-free survival.

    Article  PubMed  CAS  Google Scholar 

  15. Burdach S, Jurgens H, Peters C, et al.: Myeloablative radiochemotherapy and hematopoietic stem-cell rescue in poor-prognosis Ewing’s sarcoma. J Clin Oncol 1993, 11:1482–1488.

    PubMed  CAS  Google Scholar 

  16. Meyers P, Nachman J, Krailo M, et al.: Induction chemotherapy followed by melphalan/etoposide/total body irradiation and peripheral blood stem cell support for patients with newly diagnosed high-risk Ewing’s sarcoma. Proc Am Soc Clin Oncol 2000, 19:A2287. Consolidation with high-dose therapy with melphalan, etoposide, and total body irradiation followed by autologous stem cell reconstitution does not improve the prognosis for patients with very high-risk Ewing’s sarcoma.

    Google Scholar 

  17. Hartmann O, Pinkerton R, Dallorso G, et al.: A multivariate and matched pair analysis on high-risk Ewing Tumor (ET) patients treated by megatherapy (MGT) and stem cell reinfusion in Europe. Proc Am Soc Clin Oncol 1999, 18:A2144.

    Google Scholar 

  18. Hahm KB, Cho K, Lee C, et al.: Repression of the gene encoding the TGF-beta type II receptor is a major target of the EWS-FLI1 oncoprotein. Nat Genet 1999, 23:222–227.

    Article  PubMed  CAS  Google Scholar 

  19. Goletz TJ, Zhan S, Pendleton CD, et al.: Cytotoxic T cell responses against the EWS/FLI-1 Ewing’s sarcoma fusion protein and the PAX3/FKHR alveolar rhabdomyosarcoma fusion protein. Proc Annu Meet Am Assoc Cancer Res 1996, 37:A3243.

    Google Scholar 

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Authors and Affiliations

  1. Department of Pediatrics, Memorial Sloan-Kettering Cancer Center, 1275 York Avenue, 10021, New York, NY, USA

    Paul A. Meyers MD & Adam S. Levy MD

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  1. Paul A. Meyers MD
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  2. Adam S. Levy MD
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Meyers, P.A., Levy, A.S. Ewing’s sarcoma. Curr. Treat. Options in Oncol. 1, 247–257 (2000). https://doi.org/10.1007/s11864-000-0036-7

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  • DOI: https://doi.org/10.1007/s11864-000-0036-7

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