Léčba dětí a mladistvých s Ewingovým sarkomem vysokého stupněrizika. Zkušenost Kliniky dětské onkologie FNsP v Motole

Treatment of High Risk Ewing’s Sarcoma in Children and Adolescents. Experience of the De-partment of Paediatric Oncology, Faculty Hospital Motol

The improved survival rates have been achieved by the use of combination multicycle chemotherapy in Ewing’ssarcoma. The survival of patients presenting with a bulky non resectable tumour, detectable metastatic disease ondiagnosis or relapse is poor with only 20 - 40% disease free survival rates for three years. In an attempt to improvedthe outcome for these poor prognosis patients we explored the role of megatherapy approaches with autologousstem cell rescue. Since 1991 till 1997 26 patients with Ewing’s sarcoma/peripheral neuroectodermal tumors enteredour study. There were 18 males and 8 females, median age 11.1 years. The most common sites of the primarieswere the pelvis and vertebra, 9 children presented metastatic disease on diagnosis. In induction therapy protocolsof the German cooperative study (EICESS) (17x) and North American POG study regimes (7x) were the mostfrequently used. Preparative regimen based mostly on etoposide, carboplatinum, melphalan and/or total bodyirradiation (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. Historicalcontrol 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 highrisk 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