CPT-SIOP-2000

After maximal possible resection, all patients with Choroid Plexus carcinoma (CPC), metastasized tumors and incompletely resected atypical plexus papilloma ree randomized to a treatment with either carboplatin or cyclophosphamide given together with etoposide and vincristine. After the first interim analysis at the committee meeting in Vancouver 2005, it was decided to stratify the randomization by age to keep an even number of radiated patients in each treatment group. A total of 6 courses of chemotherapy (VP16 100 mg/m2 d1-5, vincristine 1.5 mg/m² d1 and either carboplatin 350 mg/m² d1+2 or cyclophosphamide 1000 mg/m² d1+2), and additional irradiation after the second course for those who are older than 3 years of age was given. Patients with metastasized atypical choroid plexus papilloma (APP) and CPC, and patients with non-metastasized CPC non-responsive to chemotherapy (Stable desease, Progressive desease) received craniospinal irradiation with 35 Gy and local boost up to a total of 54 Gy. Patients with choroid plexus papilloma and completely resected atypical papilloma were closely followed without further treatment.

Eligibility criteria for first registration (registration and follow-up study)

• Histological diagnosis of a choroid plexus tumor by a local pathologist / neuropathologist. This includes choroid plexus papilloma, atypical choroid plexus papilloma, anaplastic choroid plexus papilloma, malignant choroid plexus papilloma, and choroid plexus carcinoma. WHO brain tumor classification 1.5.1 and 1.5.2 (Kleihues 1993), ICD-O classification M-9390/0, M-9390/3 (WHO 1990)
• Slides have been sent to the pathology reference centre (by declaration of the sending centre)

Eligibility criteria for registration for randomization (center depended voluntary second registration or treatment study)

• The first registration on the study was completed
• The reference centre has confirmed the receipt of slides sent
• The postoperative imaging has been done and the result is available
• The indication criteria are met
• The chemotherapy start criteria are met
• The agreement of patient or legal guardian has been documented according to the local guidelines

• Patient or legal guardian does not consent to enrollment with electronic data processing or sending of tumor slides to the pathology reference centre