Atypical teratoid/rhabdoid tumours (ATRT) are rare, highly aggressive tumours of the central nervous system (brain and spinal cord). This disease primarily affects very young children aged under 22 months. Although the prospects of recovery for patients with ATRT have improved in recent years, they remain generally unfavourable despite intensive treatment strategies.
In Switzerland, about 2-3 patients a year are affected by this disease.
SIOPE ATRT01 is an international treatment optimisation study. Designed for children and adolescents, the study intends to take a decisive step towards improving treatment and cure rates while also minimising side effects. It aims to respond to certain key questions regarding the treatment of ATRT, such as whether high-dose chemotherapy followed by a stem cell transplant is not less effective than radiotherapy, but with fewer long-term complications.
Alongside medical treatment, all participants will also undergo neuropsychological assessments. A specially developed test will examine patients’ cognitive development and quality of life. These investigations are intended to help recognise, at an early stage, how the disease and its treatment affect cognitive development and enable appropriate action to be taken. The study will examine whether the cognitive development of children who received radiotherapy at an early age is more greatly affected than that of children who received high-dose chemotherapy. In the long term, the findings of these investigations will help to improve treatment methods for patients by minimising the side effects and improving the tolerability of treatment.
The University Hospital Augsburg in Germany is responsible for the international implementation of the study, with the study office and the Society of Paediatric Oncologists and Haematologists (GPOH) as sponsor. The Swiss Paediatric Oncology Group (SPOG) is responsible for performing the study in Switzerland (sponsor representative).
In short
- This study is investigating the treatment of atypical teratoid/rhabdoid tumours (ATRT), a certain type of aggressive tumours of the central nervous system in children and adolescents.
- Despite intensive treatment, the chances of recovery for patients with an ATRT are still poor.
- This study is comparing two different therapeutic approaches to find out which is most effective, and thereby optimise the treatment and improve the prospects of recovery.
