Standard Treatment Options
Treatment Options Under Clinical Evaluation
Current Clinical Trials

Standard Treatment Options

The prognosis for patients with recurrent or progressive Ewing tumor of bone (ETB) is poor, though the prognosis for patients relapsing more than 2 years following completion of primary therapy is better than for those patients who relapse while on their initial chemotherapy regimen or earlier than 2 years following completion of primary therapy.[1-3] Five-year event-free survival (EFS) and overall survival following recurrence is less than 10%.[2,4] Patients with both local and distant recurrence have a worse outcome than those with either local or distant recurrence.[1] Patients who relapse 2 or more years after diagnosis and patients who have local recurrences that can be treated with radical surgery and intensive chemotherapy have the most favorable outcomes.[1] In one large series, the majority of relapses occurred within 5 years of diagnosis. Ten percent to 15% of relapses occurred after 5 years. Very few patients who relapsed later were salvaged.[5] The selection of further treatment depends on many factors, including the site of recurrence and prior treatment, as well as individual patient considerations. Ifosfamide and etoposide are active in ETB and should be considered for patients who have not previously received these agents.[6] The combination of cyclophosphamide plus topotecan has been active in patients with recurrent or refractory disease.[7-9] The combination of irinotecan and temozolomide has achieved objective responses in patients with recurrent ETB.[10] Aggressive attempts to control the disease, including myeloablative regimens, may be warranted.[11] A European survey of patients undergoing allogeneic stem cell transplantation for recurrent ETB did not show improved EFS 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.[1] Patients with pulmonary metastases should receive whole-lung irradiation.[1] Residual disease in the lung may be surgically removed.

Clinical trials investigating new agents and new combinations of agents are available and should be considered. Information about ongoing clinical trials is available from the NCI Web site.

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. 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]
   
   
2. 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]
   
   
3. 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]
   
   
4. Bacci G, Longhi A, Ferrari S, et al.: 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 45 (4): 469-75, 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. 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]
   
   
7. 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]
   
   
8. 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]
   
   
9. 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]
   
   
10. 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]
    
    
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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