CPT-SIOP-Registry

Choroid Plexus Tumors

International Registry

Choroid plexus tumours are very rare brain tumours in which the vascular plexus in the cerebral ventricles begins to proliferate. They account for about half a percent of all brain tumours across all age groups. However, as they usually occur at an early age, the proportion in children is significantly higher, at 1 to 4% overall and up to 13% in the first year of life. In Germany, approximately 10 patients have been reported annually in recent years. A distinction is made between benign plexus papillomas, malignant plexus carcinomas and, in the middle, atypical plexus papillomas, all of which occur with approximately the same frequency. Some plexus carcinomas are caused by Li-Fraumeni syndrome, an autosomal dominant 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.

Following completion of the CPT-SIOP-2000 therapy study and prior to the launch of a follow-up study in Germany and other countries, the registry is open to all patients with plexus tumours. Given the rarity of these tumours, it remains desirable to collect as complete and comprehensive data as possible on all patients worldwide in order to ensure the best possible data quality.

Objectives

• Epidemiological and clinical recording of all patients with plexus tumours in an international database
• Structuring of central reference assessment of pathology, radiology and radiotherapy
• Scientific analysis of the recorded data
• Evaluation of survival rates (event-free and overall).
• Evaluation of the significance of surgery, radiotherapy and chemotherapy

The registry does not conduct drug testing within the meaning of the German Medicines Act (AMG). It provides advice based on the results of the CPT-SIOP 2000 trial (see completed studies). This study shows that patients with plexus carcinomas in particular can benefit from chemotherapy and, if not contraindicated by age, radiotherapy after the maximum possible removal of the tumour. Until a follow-up study is launched, patients with plexus tumours can be registered in this registry. Treatment is at the discretion of the responsible physician.

• Patients with choroid plexus tumours, confirmed by reference pathology (Neuropathology Münster, Neuropathology Bonn)
• Written informed consent of the patient or legal guardian for the collection and transfer of data