International CPT-SIOP Registry

Since June 2010, the national office for choroid plexus tumor studies of the International Society for Pediatric Oncology (SIOP) is located at the Department of Pediatric Hematology and Oncology of the University Medical Center Hamburg-Eppendorf, Germany. The CPT-SIOP Registry is open to all national and international patients with plexus tumors. Since plexus tumors are very rare, the complete and worldwide registration of all patients is still desirable to make study analysis as accurate as possible.

The choroid plexus is a highly vascularised structure in the brain which covers parts of the brain ventricles and produces the cerebrospinal fluid. Tumors of this tissue represent about 0.5% of all brain tumors; however, since these tumors tend to arise early in life, their proportion among children is significantly higher (1 to 4%), with the highest percentage (up to 13%) in the first year of life. During the last years, in Germany about 10 patients were reported to CPT-SIOP-2000 per year (about 30 worldwide). The tumors can be divided into benign choroid plexus papillomas, malignant choroid plexus carcinomas and less malignant atypical plexus papillomas. Some plexus carcinomas can be associated with the Li-Fraumeni syndrome, an autosomal dominant hereditary disorder in which the susceptibility to cancer is highly increased presumably as a result of mutations in the tumor suppressor gene TP53.

The CPT-SIOP Registry is not a clinical trial according to the German Pharmaceuticals Act (Arzneimittelgesetz, AMG).

Aims of the CPT-SIOP Registry:

• Collection of epidemiological and clinical data of all plexus tumor patients in an international database
• Quality assurance via central review by the reference centers for histopathology, imaging and radiation therapy within the HIT treatment network
• Scientific analysis of the collected data

The study office is supported by the German Childhood Cancer Foundation ( www.kinderkrebsstiftung.de ). For counselling regarding diagnostics and treatment, please contact us via e-mail.

For more information, see the links above on the right side and:
http://www.kinderkrebsinfo.de/e1676/e9032/e1758/e92209/index_ger.html