Author:  Dr. med. U. Kordes, Dr. rer. nat. S. Hartung, Last modification: 2024/03/21 https://kinderkrebsinfo.de/doi/e92209


International CPT-SIOP-Registry


Choroid Plexus Tumors


International Registry

Problem / Objectives

Choroid plexus tumors are very rare tumours. They arise from normal tissue in the brain which produces cerebral spinal fluid, the "choroid plexus". Choroid plexus tumors account for only 0.5% of all tumors in adults and children. Most of the tumors occur in infants, but they can occur at any age. In children the tumors account between 1% and 4%, in children younger than one year they account up to 13%. In Germany over the last years ca. 13 patients per year were registered.
There are three tumor types, occuring with the same frequency: choroid plexus papilloma (relatively benign), atypical choroid plexus papilloma (intermediate form) and choroid plexus carcinoma (malignant). In some choroid plexus carcinoma a Li-Fraumeni-syndrome can be found, a rare autosomal dominant cancer predisposition, where the gene p53 is dysfunctioned. In other patients can be found an increase of the expression and copynumber of a gene for PDGF-receptors.

After the end of the trial CPT-SIOP-2000, which is still to be analized finally, and before the opening of the next trial CPT-SIOP 2009 in germany and other countries, the registry is open for all patients. Given the rareness of the desease the collection of the data of all patients over the world is desirable.


  • Epidemiological and clinical recruitment of all patients with Choroid-Plexus tumors into an international database
  • Structured reference expertise for pathology, radiology and radiotherapy
  • Scientific analysis of all collected data
  • Evaluation of survival rates
  • Evaluation of the signifincance of the surgery, radiotherapy and chemotherapy
Therapy / Study arms

The registry is not a clinical trial. It provides advice on basis of the interim-analysis of the trial CPT-SIOP 2000. The CPT-SIOP 2000 shows that patients with a choroid plexus carcinoma profit from a maximal possible surgical resection and irradiation, at least in children old enough that it can be given.
The CPT-SIOP-2009 Study will address the question, which of four different chemotherapy protocols is superior. Until the opening of the new study, all patients should be registered in the registry. The responsible doctor of the clinic decides over the treatment plan.

Inclusion Criteria
  • Patients with a Choroid-Plexus tumor, proven by a reference pathologist (Neuropathology Münster or Bonn)
  • Written informed concense of the patient or guardian for the collection and transfer of data
Recruitment about 30 per year worldwide (about 10 per year in Germany)
Status 03/10 until start of the new study
Principal Investigator Dr. med. D. Obrecht-Sturm
E-Mail cpt@uke.de
URL http://www.uke.de/cpt

Local chair

Dr. med. Denise Obrecht-Sturm Universitätsklinikum Hamburg Eppendorf Klinik und Poliklinik für Pädiatrische Hämatologie und Onkologie Martinistr. 52 20246 Hamburg Telefon +49 (0)40 7410-52741 d.obrecht@uke.de

Trial Coordination

Dr.rer. nat. Stefan Hartung Universitätsklinikum Eppendorf Klinik und Poliklinik für päd. Onkologie/Hämatologie, CPT-Register Martinistr. 52 20246 Hamburg Telefon +49 040 7410 57697 Fax +49 040 7410 57544 s.hartung@uke.de

Link(s) Literature on CPT Documents für the CPT-Registry
Sponsoring Deutsche Kinderkrebsstiftung