Clinical management of brain stem glioma.

Brain and spinal tumours in childhood (0–15 years) account for 20–25% of childhood cancer, aVecting one in 2500 children. In the UK, this means that about 350 new cases are diagnosed each year, of which 55% and 50% survive five and 10 years, respectively. For those who are survivors, about 60% have cognitive deficits and 20–30% have diYculties with mobility and chronic pain. Deaths from this group of tumours in England and Wales account for the loss of over 10 000 life years each year (C Stiller, personal communication, 1995). Within the group of brain and spinal tumours there are a number of well defined categories with characteristic clinical presentations, biological behaviour, and suitability for specific treatment approaches. These factors combine to predict a range of outcomes from the highly curable tumours (> 80% 10 year survival rate) such as germinoma and cerebellar astrocytoma, through to the virtually incurable brain stem glioma. Age at presentation is also a crucial factor aVecting both prognosis and treatment selection, especially for those who develop tumours early in life before the brain has completed growth and development. Brain stem gliomas account for about 10% of all brain and spinal tumours. The selection of brain stem glioma for this article was prompted by diYculties surrounding its clinical diagnosis, both professional and lay uncertainty about optimal surgical management, ineVectiveness of non-surgical treatment, clinical diYculties with good palliative care, and a historical lack of clear guidance about optimal referral patterns to children’s cancer centres, where appropriate resources have already been centralised for children’s cancers of other organs. 5 We define the brain stem as extending from the midbrain (tectal plate) to the medullary cervical junction (fig 1A). Therefore, brain stem glioma is a term describing a collection of anatomically related tumours with characteristic appearances on computed tomography (CT) and magnetic resonance imaging (MRI) scans. Because they are frequently not biopsied, “glioma” is used as a “catch all” term unless a histological diagnosis has been made.