Management of choroid plexus tumours in children: 20 years experience at asingle neurosurgical centre

Objective: Tumours of the choroid plexus are rare tumours of neuro-ectoderm al origin, accounting for less than 1% of all intracranial tumours. Most ca ses present in children less than 2 years of age. While choroid plexus carc inomas (CPC) are reported to have an extremely poor prognosis, choroid plex us papillomas (CPP) are generally regarded as benign tumours with a very fa vourable long-term outcome. Management dilemmas are associated with the cho ice of surgical procedure, tumour vascularity, the treatment of hydrocephal us and the value of adjuvant therapy. The objective of this study was to re view our experience with this rare tumour over a 20-year period. Methods Pa tients were identified from the Great Ormond Street Neurosurgical Brain Tum our Database. Over a 20-year period (1979-1999), 34 children were identifie d with a choroid plexus tumour. There were 25 cases of CPP and 9 cases of C PC. A retrospective review of case notes, radiological imaging, operation r eports and pathology was performed. Results: The median age at presentation was 17 months (1-138) for CPP and 13 months (2-102) for CPC. There was no sex difference for CPP. However, 8 of the 9 CPCs were male (89%). A complet e surgical resection was achieved in all 25 cases of CPP and in 3 cases of CPC (33%). The median survival for CPPs was 75.5 months (2-228), with a med ian follow-up of 73.5 months (2-228). The median survival for CPCs was 6 mo nths (1-90), with a median follow-up of 6 months (1-90). Conclusion With mo dern neurosurgical practise, a cure should be the aim for all children with CPP. There is no evidence that adjuvant therapy has any role in the primar y management of these children. However, CPC still has an extremely poor pr ognosis, and the efficacy of adjuvant therapy remains to be established. Co pyright (C) 2000 S. Karger AG. Basel.