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Diagnostic and therapeutic strategies for children and adolescents with Ewing's sarcoma/PNET

Publication at First Faculty of Medicine, Second Faculty of Medicine |
2006

Abstract

We evaluate the therapeutic results in patients with Ewing's sarcoma tumours. Methods: In 1997 through 2003 we admitted 56 patients (28 girls and 28 boys) with this diagnosis.

Their average age was 12.8 years (2-23). Localised disease was diagnosed in thirty nine patients (70%) while 17 patients (30%) had metastatic disease.

We used protocols POG 9354/CCG7942 for 34 patients and EURO-Ewing 99 for 22 patients, respectively. Megachemotherapy was given in 31 patients.

Results: Forty four patients achieved either complete remission or very good partial response. Fourteen suffered relapses.

Twenty one patients died - 5 progressed, 13 relapsed and 3 died due to treatment related toxicity. The five year EFS was 56% (68% for the localised and 26% for the metastatic disease) with the median follow up of 2,5 years (1-7 years).

Patients treated with POG 9354/CCG7942 had a 5 year EFS 51% with a median follow up of 4,1 years. The EURO Ewing 99 group had a 3 year EFS of 72% with a median follow up of 1,9 years.

Conclusion: The results of treatment of localised and even inoperable tumours were very good. The prognosis for patients with metastatic disease (especially extra-pulmonary metastases) remains very poor in spite of intensive treatment.

For these patients we need to find new methods of treatment.