Purpose of review: Primary central nervous system malignancies incorporate a variety of tumours with diverse biology and clinical behaviour and represent the most common solid tumour entity of childhood, accounting for approximate 20-25% of all primary paediatric malignancies. Recent findings regarding the underlying tumour biology may open up new avenues of clinical trial design, particularly identifying possible targets for biological modifiers. Over the last 12-18 months a significant number of institutional and national studies have been reported which are likely to impact on the design of future clinical trials.
Recent findings: In low-grade gliomas, stereotactically guided conformal radiotherapy should lead to a significant reduction in radiation-associated late toxicity, while in selected groups of high-grade gliomas the use of adjuvant or neo-adjuvant chemotherapy may improve survival. Completeness of resection and use of adjuvant focal radiotherapy remains the most important prognostic factor for outcome in patients with ependymomas, although in infants the use of post-surgical chemotherapy alone may allow the postponing of radiotherapy in selected cases. In primitive neuroectodermal tumours prognostic biological markers have been identified that are undergoing prospective evaluation. For patients with localized medulloblastomas a new standard treatment is emerging that uses reduced-dose craniospinal radiotherapy followed by platinum-based chemotherapy, while in supratentorial primitive neuroectodermal tumours future treatment will be aimed at improving local control.
Summary: Given the rarity of paediatric primary central nervous system malignancies, further progress can only be achieved in the context of national or multinational prospective clinical trials incorporating biological studies, and participation in these should be strongly encouraged.
Copyright 2002 Lippincott Williams & Wilkins