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The importance of radiotherapy in paediatric atypical teratoid rhabdoid tumour of the brain
open access
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.
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.
Keywords
atypical teratoid rhabdoid tumour; children; radiotherapy


Title
The importance of radiotherapy in paediatric atypical teratoid rhabdoid tumour of the brain
Journal
Reports of Practical Oncology and Radiotherapy
Issue
Pages
53-57
Published online
2009-03-01
DOI
10.1016/S1507-1367(10)60022-3
Bibliographic record
Rep Pract Oncol Radiother 2009;14(2):53-57.
Keywords
atypical teratoid rhabdoid tumour
children
radiotherapy
Authors
Elżbieta KORAB CHRZANOWSKA
Joanna BARTOSZEWSKA
Monika DROGOSIEWICZ
Stanisław KWIATKOWSKI
Anna SKOWROŃSKA-GARDAS