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, |
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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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