Klin Onkol 1999; 12(4): 120-123.
Summary: The prognosis of patients with either localized Ewing´ s sarcomas or malignant peripheral neuroectodennal tumors has improved with systemic combination chemotherapy and local control with surgery and/or radiation therapy, and is ranges between 50 - 70% disease free survival at five years. Despite these improvements, survival for those with large, inoperable axial primaries, distant metastases or relapse remains disappointing, with less than 20% of patients surviving more than three years. In an attempt to improve the outcome for these poor prognosis patients, we explored the role of consolidation therapy with megatherapy and autologous hematopoietic rescue. From 1991 to 1998,27 patients with Ewing´s sarcoma and 4 with peripheral neuroectodennal tumors received megatherapy. There were 20 males and 11 females, median age 12.6 years. Twenty two achieved first or second complete or very good remission at the time of transplantation, 7 second partial remission and two had progressive disease. The reparative regimen contained etoposide and carboplatine, melphalan and total body irradiation in some cases. Overall survival 58% (19 month follow up). Thirteen patients died: 11 by disease progression, one by infectious complications and secondary myelodysplastic syndrome developed in one female with lethal outcome. Median time to relapse after megatherapy was 9.3 months. In conclusion, there is evidence that myeloablative therapy has improved the outcome in at least at some patients with high risk Ewing´s sarcoma. The exact role of megatherapy remains to be more precisely determined.
