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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. We take care of follow-up reports to the expired CPT-SIOP-2000 study (also for international patients), are involved in the final analysis of this study and in the preparations for the conduct of the successor study CPT-SIOP-2009 in Germany. In the meantime, 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 wordwide). 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. Also, in many plexus carcinomas an increase in expression and copy number of genes for PDGF receptors can be found.
The CPT-SIOP Registry is not a clinical trial in the sense of the German Pharmaceuticals Act (Arzneimittelgesetz). It provides counseling based on the interim results of the CPT-SIOP-2000 study, which show that especially patients with plexus carcinomas can benefit, after resection of the tumor, from chemotherapy and, if old enough, from radiation therapy. In the successor study CPT-SIOP-2009, four treatment arms shall be compared.
Aims of the CPT-SIOP Registry:
- Collection of epidemiological and clinical data of all plexus tumor patients in an international database
- Assurance of central reviewing by the reference centers for histopathology, imaging and radiation therapy within the HIT treatment network
- Scientific analysis of the collected data
- Determination of survival rates (overall and event-free)
- Determination of the relative influences of surgery, radiation and chemotherapy on survival
The study office is supported by the German Childhood Cancer Foundation (www.kinderkrebsstiftung.de).
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