Abstract

Brain stem and cervicomedullary tumours are typical of paediatric age, 80% of them occurring in patients under 18 years of age, and comprising 10–15% of all childhood and adolescent brain tumours, as well as 20–25% of infratentorial locations.They are characteristically pontine tumours (60% of the cases), but they commonly extend to involve the medulla, midbrain and cerebellum. Although most brain stem tumours are low grade gliomas, their prognosis is extremely severe (no more than 20% of patients are alive 3 years after diagnosis and the 5-year survival rate is 5%) because both the typical infiltrating nature and the neuro-anatomical location usually make them surgically unresectable. Surgery is generally limited to biopsy, partial decompression, or excission of the exophytic components, because of the extremely severe functional sequelae of even minor resections. Thus, the mainstay of therapy has been based on irradiation alone or combined with chemotherapy, doses of 5000–5500 cGy being usually adequate...

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