Germ cell tumours of the central nervous system in children-controversies in radiotherapy

BACKGROUND: Primary germ cell tumours (GCT) of the central nervous system (CNS) are rare tumours of children and adults. As few prospective, randomised trials have been published, management decisions are often based on retrospective and historical studies, histology, age, performance status, and extent of disease at diagnosis. This article reviews the current controversies in the radiotherapeutic management of primary GCT of the CNS. PROCEDURE: A computerised literature search was performed using the Medline database from 1975 to 2000, this being limited to publications written in the English language on CNS GCT in children up to the age of 16 years. RESULTS: Issues in radiation therapy currently undergoing review include the role of cranio-spinal irradiation (CSI), radiation therapy field sizes, the radiation therapy dose, the extent of surgery in combination with radiotherapy, and the role and timing of chemotherapy in combination with radiotherapy. CONCLUSIONS: The excellent outcome in children with pure germinoma of the CNS and the long-term morbidity associated with standard therapy justifies attempts to limit both the total radiation dose and field sizes, with or without the addition of chemotherapy. The poorer outcome associated with non-germinoma GCT justifies a more aggressive approach in children, combining chemotherapy, surgery and virgule, or radiation therapy, based on known prognostic factors.