Standard Treatment Options
Treatment Options Under Clinical Evaluation
Current Clinical Trials

Standard Treatment Options

Recurrence of Ewing tumor of bone (ETB) is most common within 2 years of initial diagnosis (approximately 80%); late recurrence beyond 5 years occurs in approximately 10% of patients.[1][Level of evidence: 3iiA] The prognosis for patients with recurrent ETB is poor; 5-year survival following recurrence is approximately 10% to 15%.[1-4][Level of evidence: 3iiA] Time to recurrence is an important prognostic factor. Patients who recurred greater than 2 years from initial diagnosis had a 5-year survival of 30% versus 7% for patients who recurred within 2 years.[1][Level of evidence: 3iiA] Patients with both local recurrence and distant metastases have a worse outcome than patients with either isolated local recurrence or metastatic recurrence alone.[1,2][Level of evidence: 3iiA] Isolated pulmonary recurrence was not an important prognostic factor.[1][Level of evidence: 3iiA] Ten percent to 15% of relapses occurred after 5 years. Very few patients who relapsed later were salvaged.[5]

The selection of treatment for patients with recurrent disease depends on many factors, including the site of recurrence and prior treatment, as well as individual patient considerations. Combinations of chemotherapy such as cyclophosphamide, topotecan or irinotecan, and temozolomide are active in recurrent Ewing family of tumors and can be considered for these patients.[6-9] Ifosfamide and etoposide may be active in patients who have not previously received these therapies.[10] Aggressive attempts to control the disease, including myeloablative regimens, have been used but there is no evidence at this time to conclude that myeloablative therapy is superior to standard chemotherapy.[11] A European survey of patients undergoing allogeneic stem cell transplantation for recurrent ETB did not show improved event-free survival when compared with autologous stem cell transplantation and was associated with a higher complication rate.[12] Radiation therapy to bone lesions may provide palliation, though radical resection may improve outcome.[2] Patients with pulmonary metastases should receive whole-lung irradiation.[2] Residual disease in the lung may be surgically removed.

The following is an example of a national or international clinical trial that is currently being conducted. For more information about clinical trials, please see the NCI Web site

• COG-AEWS-0521: The Children's Oncology Group (COG) is conducting a randomized phase II trial of a novel antiangiogenic agent bevacizumab in combination with standard salvage chemotherapy. Patients experiencing their first recurrence of Ewing sarcoma will be randomized to receive vincristine, topotecan, and cyclosphosphamide with or without bevacizumab.

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with recurrent Ewing sarcoma/peripheral primitive neuroectodermal tumor. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.

General information about clinical trials is also available from the NCI Web site.

References

1. Leavey PJ, Mascarenhas L, Marina N, et al.: Prognostic factors for patients with Ewing sarcoma (EWS) at first recurrence following multi-modality therapy: A report from the Children's Oncology Group. Pediatr Blood Cancer 51 (3): 334-8, 2008.  [PUBMED Abstract]
   
   
2. Rodriguez-Galindo C, Billups CA, Kun LE, et al.: Survival after recurrence of Ewing tumors: the St Jude Children's Research Hospital experience, 1979-1999. Cancer 94 (2): 561-9, 2002.  [PUBMED Abstract]
   
   
3. Shankar AG, Ashley S, Craft AW, et al.: Outcome after relapse in an unselected cohort of children and adolescents with Ewing sarcoma. Med Pediatr Oncol 40 (3): 141-7, 2003.  [PUBMED Abstract]
   
   
4. Bacci G, Longhi A, Ferrari S, et al.: Pattern of relapse in 290 patients with nonmetastatic Ewing's sarcoma family tumors treated at a single institution with adjuvant and neoadjuvant chemotherapy between 1972 and 1999. Eur J Surg Oncol 32 (9): 974-9, 2006.  [PUBMED Abstract]
   
   
5. Bacci G, Forni C, Longhi A, et al.: Long-term outcome for patients with non-metastatic Ewing's sarcoma treated with adjuvant and neoadjuvant chemotherapies. 402 patients treated at Rizzoli between 1972 and 1992. Eur J Cancer 40 (1): 73-83, 2004.  [PUBMED Abstract]
   
   
6. Saylors RL 3rd, Stine KC, Sullivan J, et al.: Cyclophosphamide plus topotecan in children with recurrent or refractory solid tumors: a Pediatric Oncology Group phase II study. J Clin Oncol 19 (15): 3463-9, 2001.  [PUBMED Abstract]
   
   
7. McTiernan A, Driver D, Michelagnoli MP, et al.: High dose chemotherapy with bone marrow or peripheral stem cell rescue is an effective treatment option for patients with relapsed or progressive Ewing's sarcoma family of tumours. Ann Oncol 17 (8): 1301-5, 2006.  [PUBMED Abstract]
   
   
8. Hunold A, Weddeling N, Paulussen M, et al.: Topotecan and cyclophosphamide in patients with refractory or relapsed Ewing tumors. Pediatr Blood Cancer 47 (6): 795-800, 2006.  [PUBMED Abstract]
   
   
9. Wagner LM, McAllister N, Goldsby RE, et al.: Temozolomide and intravenous irinotecan for treatment of advanced Ewing sarcoma. Pediatr Blood Cancer 48 (2): 132-9, 2007.  [PUBMED Abstract]
   
   
10. Miser JS, Kinsella TJ, Triche TJ, et al.: Ifosfamide with mesna uroprotection and etoposide: an effective regimen in the treatment of recurrent sarcomas and other tumors of children and young adults. J Clin Oncol 5 (8): 1191-8, 1987.  [PUBMED Abstract]
    
    
11. Burdach S, Jürgens H, Peters C, et al.: Myeloablative radiochemotherapy and hematopoietic stem-cell rescue in poor-prognosis Ewing's sarcoma. J Clin Oncol 11 (8): 1482-8, 1993.  [PUBMED Abstract]
    
    
12. Burdach S, van Kaick B, Laws HJ, et al.: Allogeneic and autologous stem-cell transplantation in advanced Ewing tumors. An update after long-term follow-up from two centers of the European Intergroup study EICESS. Stem-Cell Transplant Programs at Düsseldorf University Medical Center, Germany and St. Anna Kinderspital, Vienna, Austria. Ann Oncol 11 (11): 1451-62, 2000.  [PUBMED Abstract]
    
    

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