Skip to content
My WebMD Sign In, Sign Up

Cancer Health Center

Font Size

Ewing Sarcoma Treatment (PDQ®): Treatment - Health Professional Information [NCI] - Ewing Sarcoma: Recurrent Tumors

Standard Treatment Options

Recurrence of Ewing sarcoma is most common within 2 years of initial diagnosis (approximately 80%).[1,2] However, late relapses occurring more than 5 years from initial diagnosis are more common in Ewing sarcoma (13%; 95% confidence interval, 9.4–16.5) than in other pediatric solid tumors.[3] The overall prognosis for patients with recurrent Ewing sarcoma is poor; 5-year survival following recurrence is approximately 10% to 15%.[2,4,5]; [1][Level of evidence: 3iiA] Time to recurrence is the most 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.[2]; [1] 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] Isolated pulmonary recurrence was not an important prognostic factor.[1]

Recommended Related to Cancer

Treatment Options for Refractory Chronic Lymphocytic Leukemia

Treatment of refractory chronic lymphocytic leukemia may include the following: A clinical trial of chemotherapy with stem cell transplant. A clinical trial of a new treatment. Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with refractory chronic lymphocytic leukemia. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General...

Read the Treatment Options for Refractory Chronic Lymphocytic Leukemia article > >

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 and topotecan or irinotecan and temozolomide, are active in recurrent Ewing sarcoma and can be considered for these patients.[6,7,8,9,10] There is no standardized second-line treatment for relapsed or refractory Ewing sarcoma. One phase II study of topotecan and cyclophosphamide showed a response in 6 of 17 patients with Ewing sarcoma; 16 of 49 patients had a clinical response in a similar trial from Germany.[6,8] In one retrospective series, 20 patients received temozolomide and irinotecan following recurrence. Five patients achieved a complete response and seven patients achieved a partial response.[10] The combination of gemcitabine and docetaxel has achieved objective responses in relapsed Ewing sarcoma.[11][Level of evidence: 3iiiDiv] High-dose ifosfamide (3 g/m2 /day for 5 days = 15 g/m2) has shown activity in patients who recurred after therapy which included standard ifosfamide (1.8 g/m2 /day for 5 days = 9 g/m2).[12][Level of evidence: 3iiiDiv]

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.[13,14]; [15][Level of evidence: 3iiiDiii] Surveys of patients undergoing allogeneic stem cell transplantation for recurrent Ewing sarcoma did not show improved event-free survival when compared with autologous stem cell transplantation and was associated with a higher complication rate.[13,16,17]

Monoclonal antibodies against the insulin-like growth factor 1 receptor (IGF1R) are reported to produce objective responses in metastatic recurrent Ewing sarcoma in roughly 10% of cases.[18,19,20,21][Level of evidence: 3iiDiv] In these studies, it was suggested that time-to-progression was prolonged compared with historical controls. Further studies are needed to identify patients who are likely to benefit from IGF1R therapy.

1|2

WebMD Public Information from the National Cancer Institute

Last Updated: February 25, 2014
This information is not intended to replace the advice of a doctor. Healthwise disclaims any liability for the decisions you make based on this information.
Next Article:

Today on WebMD

Building a Support System
Blog
cancer fighting foods
SLIDESHOW
 
precancerous lesions slideshow
SLIDESHOW
quit smoking tips
SLIDESHOW
 
Jennifer Goodman Linn self-portrait
Blog
what is your cancer risk
HEALTH CHECK
 
colorectal cancer treatment advances
Video
breast cancer overview slideshow
SLIDESHOW
 
prostate cancer overview
SLIDESHOW
lung cancer overview slideshow
SLIDESHOW
 
ovarian cancer overview slideshow
SLIDESHOW
Actor Michael Douglas
Article