CPT SIOP

PURPOSE: Choroid plexus tumors - papillomas and atypical papillomas (CPP) and carcinomas (CPP) are rare tumors originating from choroid plexus. The incidence is highest in children. Treatment, as it is common in other rare tumors, depends on a specification of diagnosis and experience collected from a case by case method and by global effort of many specialists. The International Registry of Choroid Plexus Tumors allows that as a powerful tool for diagnosis, treatment, incidence and survival information and improving the care by supporting prospective randomized protocols, follow-ups and literature monitoring. METHOD: 233 patients with choroid plexus tumors were registered from 173 nations from January 2000 until now. Reference pathology was completed in more than 150 cases. RESULTS: The projected 5-year overall survival in CPP is 100 % and event free survival ( EFS) 88%, OS in APP is 91% and EFS in APP is 79%, OS in CPC is 48% and EFS in CPC is 43%. The outcome in patients that received radiation is better than of those without radiation. The effect of surgery cannot be detected any more- patients with partial resection do as well as those with complete resection. By CPT-SIOP-2000 chemotherapy makes no difference in the outcome compare to other treatments - more detailed analysis is warranted for CPT SIOP2012 Meeting. The protocol CPT-SIOP-2009 has opened in Boston, but the accrual is slow. CONCLUSION: While there is overlap CPT 2000 and CPT 2009 Registries, the next protocol CPT-SIOP 2009 should be opened in more centers and opening of new world-wide centers is being encouraged