Recurrence of Ewing tumor of bone (ETB) is most common within 2 years of initial diagnosis (approximately 80%). However, late relapses occurring more than 5 years from initial diagnosis are more common in ETB (13%; 95% confidence interval, 9.4-16.5) than in other pediatric solid tumors. The overall prognosis for patients with recurrent Ewing sarcoma is poor; 5-year survival following recurrence is approximately 10% to 15%.[3,4,5]; [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.[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.; [Level of evidence: 3iiA] Isolated pulmonary recurrence was not an important prognostic factor.[Level of evidence: 3iiA]
Routine cancer screening can save lives. It can also cause serious harm.
This is the "double-edged sword" of cancer screening, says Otis Webb Brawley, MD, chief medical officer at the American Cancer Society.
"Many of these cancers we treat and cure never needed to be treated and cured," Brawley says. "They are never going to kill that patient."
At the heart of the problem is our justifiable fear of cancer. The message has been drummed into us: Find cancers early while they're still curable and...
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 family of tumors and can be considered for these patients.[6,7,8,9,10] In one series, 20 patients received temozolomide and irinotecan following recurrence. Five patients achieved a complete response and seven achieved a partial response. The combination of gemcitabine and docetaxel has achieved objective responses in relapsed Ewing sarcoma.[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).[Level of evidence: 3iiiDiv] Ifosfamide and etoposide may be active in patients who have not previously received these therapies.
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.[14,15]; [Level of evidence: 3iiiDiii] Surveys 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.[14,17,18]
Radiation therapy to bone lesions may provide palliation, though radical resection may improve outcome. Patients with pulmonary metastases who have not received radiation therapy to the lungs should be considered for whole-lung irradiation. Residual disease in the lung may be surgically removed.
Treatment Options Under Clinical Evaluation
The following is an example of a national or international clinical trial that is currently being conducted. Information about ongoing clinical trials is available from the NCI Web site
COG-ADVL0918: A phase I trial combining temsirolimus with irinotecan and temozolomide in children, adolescents, and young adults with relapsed or refractory solid tumors.