Outcomes of choroid plexus tumours diagnosed in the North West region of the UK during 1954-2008

Background: Choroid plexus tumours (CPTs); namely choroid plexus papilloma (CPP) and choroid plexus carcinoma (CPC) are rare neoplasms of the central nervous system (CNS), accounting for 0.4-0.6% of all brain tumours and 1-4% of childhood brain tumours. This study was undertaken to provide further insight into the epidemiology, natural history and outcomes in relation to changes in treatment emphasis for these rare tumours, over the last 56 years in the NW of England and determine the relative importance of possible contributory factors.Methods: Data relating to presentation, management and survival for 57 patients diagnosed with CPT between 1954-2008 was extracted from the North West Children’s Tumour Registry and hospital records.Results: Data collated showed a male predilection for choroid plexus carcinomas (22 male, 6 female) and an equal distribution of CPP and CPC (29 and 28 respectively). 5 year Kaplan-Meier estimates demonstrate significant improvements in survival for papillomas over the last 56 years with extent of resection being the most important factor in survival (p=0.001). Carcinomas show no significant improvement in survival over the last 56 years with 5 year survival fluctuating by 10% over the last 40 years. However, overall survival is improved by adjuvant treatment with both chemotherapy and radiotherapy (p