The registry does not conduct drug trials within the meaning of the AMG. It provides advice based on the interim results of the CPT-SIOP-2000 study. This shows that patients with plexus carcinomas in particular can benefit from chemotherapy and, if not contraindicated by age, radiotherapy after the greatest possible removal of the tumour. Until a follow-up study is opened, patients with plexus tumours can be registered in this registry. Treatment is at the discretion of the responsible physician.
Choroid plexus tumours are very rare brain tumours in which the choroid plexus in the ventricles of the brain begins to proliferate. They account for around half a percent of all brain tumours across all age groups. However, as they usually occur at an early age, the overall proportion in children is significantly higher at 1 to 4%, and even up to 13% in the first year of life. In Germany, around 10 patients a year have been reported in recent years. A distinction is made between benign plexus papillomas, malignant plexus carcinomas and, in the middle position, atypical plexus papillomas, all of which occur with approximately the same frequency. Some plexus carcinomas are caused by Li-Fraumeni syndrome, an autosomal-dominantly inherited tumour predisposition in which the tumour suppressor gene TP53 is usually altered. Furthermore, the expression and copy number of one of the genes for PDGF receptors is often increased.
After completion of the CPT-SIOP-2000 therapy study, which is currently in the evaluation phase, and before the opening of a follow-up study in Germany and other countries, the registry will be open to all patients with plexus tumours. In view of the rarity of the tumours, it is still desirable for the best possible data quality that all patients are recorded as completely as possible worldwide.
