The Swiss Paediatric Oncology Group SPOG is providing access to a study that aims to improve treatment of a rare brain tumour in children. The study hopes to mitigate the late complications of treatment.
Atypical teratoid rhabdoid tumours (AT/RT) are a rare, difficult-to-treat type of brain tumour. Just one or two cases occur in Switzerland each year, usually in infants and very young children. Since survival rates are generally low, action is urgently required to improve treatment.
The standard treatment for AT/RT is surgical removal of the tumour, chemotherapy and, if the patient is of suitable age, radiotherapy. If surgeons are able to completely remove the tumour and there are no metastases, patients aged three or over only receive radiotherapy at the tumour site. However, even this involves the risk of severe acute side effects at a later date. Longer-term side effects are primarily restricted cognitive development and neuroendocrine dysfunction (disrupted hormone levels).
The SIOPE ATRT01 study is therefore investigating whether the conventional combination of radiotherapy and chemotherapy can be replaced by high-dose chemotherapy alone followed by stem cell transfusion in children aged 12 to 35 months who are in the consolidation phase, during which doctors try to prevent the tumour returning. The aims of the study are firstly to reduce the longer-term side effects of treatment and secondly to improve the survival rate among children with AT/RT. Children under the age of 12 months will receive high-dose chemotherapy followed by stem cell transfusion, while children over the age of 36 months will receive a combination of chemotherapy and radiotherapy.
Further information on the SIOPE ATRT01 study.
« Taking part in the study is extremely important for the affected children because it may be possible to dispense with radiotherapy and mitigate complications at a later date, particularly as regards neurocognition. »
Prof. Katrin Scheinemann
Head of Paediatric Oncology and Haematology, Cantonal Hospital Aarau
