Intrinsic brainstem tumours in childhood: a report of 35 children followed for a minimum of 5 years.

The presentation, pathology and outcome of 35 children with intrinsic brainstem tumours treated at our institution between 1980 and 1990 have been reviewed. Eight children remain alive. There were 19 girls and 16 boys. Mean age at diagnosis was 5 years. The mean duration of symptoms prior to diagnosis was 5 months. The diagnosis was made by computed tomography (CT) in all children and magnetic resonance imaging (MRI), in addition, for 24 children. Twenty-seven tumours (77%) were diffuse, three (8.6%) were predominantly cystic, three (8.6%) were exophytic and two (5.7%) were focal. Cervicomedullary tumours were not included in this study. Definitive histology was obtained in 13 children (37.1%), eight (22.9%) by biopsy alone and five (14.3%) after surgery to debulk the tumours. Eleven of these cases were astrocytomas and two were primitive neuroectodermal tumours (PNET). Twelve children (34.3%) required shunt placement for hydrocephalus. Twenty-eight children (80%) were irradiated using conventionally fractionated radiotherapy to a total dose between 50 and 55 Gy. Six children (17.1%) also received chemotherapy. The overall mean survival time was 10 months, but five of six children who had their tumours debulked survived free of progressive disease for more than 5 years following their diagnosis. The prognosis for brainstem tumours in children remains poor with only a minority falling into the surgically resectable (good prognosis) group.