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  Česky / Czech version Čes.-slov. Pediat., 55, 2000, No. 5, p. 300-304.
 
Treatment of High Risk Ewing’s Sarcoma in Children and Adolescents. Experience of the De- partment of Paediatric Oncology, Faculty Hospital Motol 
Mališ J. 1 , Kavan P. 1 , Kabíčková E. 1 , Jeřábková V. 1 , Válková J. 1 , Stejskalová S. 1 , 

Klinika dětské onkologie 2. LF UK, FNsP v Motole, Praha,
 


Summary:

       The improved survival rates have been achieved by the use of combination multicycle chemotherapy in Ewing’s sarcoma. The survival of patients presenting with a bulky non resectable tumour, detectable metastatic disease on diagnosis or relapse is poor with only 20 - 40% disease free survival rates for three years. In an attempt to improved the outcome for these poor prognosis patients we explored the role of megatherapy approaches with autologous stem cell rescue. Since 1991 till 1997 26 patients with Ewing’s sarcoma/peripheral neuroectodermal tumors entered our study. There were 18 males and 8 females, median age 11.1 years. The most common sites of the primaries were the pelvis and vertebra, 9 children presented metastatic disease on diagnosis. In induction therapy protocols of the German cooperative study (EICESS) (17x) and North American POG study regimes (7x) were the most frequently used. Preparative regimen based mostly on etoposide, carboplatinum, melphalan and/or total body irradiation (3 cases). Except one in all the patients grafted. With a median time of follow up 3.9 years 17 children (65%) are overall survival, 15 (58%) remain disease free. Nine patients died: 7 by disease progression. Historical control group of 34 patients is statistically comparable. All the children were treated by induction therapy, radiotherapy and/or surgery. Seven patients (24%) are alive, four of them disease free survival. In conclusion, there is statistically significant evidence (p = 0.004) that myeloablative therapy has an improved outcome for high risk Ewing’s sarcoma patients in our institution. The exact role of megatherapy need to be further investigated.

        Key words: Ewing’s sarcoma, PNET, high risk, pelvis, megatherapy
       

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