The importance of radiotherapy in paediatric atypical teratoid rhabdoid tumour of the brain

Abstract

Background

Atypical teratoid rhabdoid tumours (ATRT) are very rare children's cancers. Approximately 200 cases of ATRT located in the central nervous system have been described in the literature up till now.

Aim

The aim of this report was to analyze the results of treatment of 8 children with these very rare neoplasms of the central nervous system, who were treated according to the Polish Paediatric Brain Tumour Group protocol.

Material and Methods

Eight children aged from 4 months to 22 years, 5 girls, 3 boys with ATRT of the central nervous system are presented. All children have been operated on and received multidrug chemotherapy; 5 children received radiotherapy as well. In all craniospinal irradiation was applied, in doses of 35 Gy to the whole axis and 55 Gy to tumour boost.

Results

Five patients died and 3 children are still alive. The progression-free survival of all 8 patients was 3 to 73 months. The overall survival was 5 to 73 months. All living children received radiotherapy. Two of them had total surgical resection and one partial.

Conclusions

We conclude that radiotherapy prolonged survival in ATRT and should be incorporated in all treatment protocols for patients with this diagnosis.