Clinical features, treatment and functional prognosis of infants with CNS tumors

Central Nervous System (CNS) tumours in children under 3 years of age make up 1/3 of all cases of paediatric brain tumours. At this age prognosis is worse compared to older children due to several factors such as prevalence of more aggressive oncotypes (AT/RT, Choroid Plexus Carcinoma) or more aggressive behaviour within the same tumour type, higher surgical risk, limitations in therapeutic measures (radiotherapy). Delay in diagnosis is common due to cranial vault plasticity which allows a better tolerance to intracranial hypertension. Since 1985 a series of studies (both monocentre and cooperative) have been conducted in order to delay or omit irradiation and, at the same time, increase the chance of cure. Although the goal of omitting or delaying radiotherapy has not been reached in the majority of patients, we learned how to intensify chemotherapy to reduce tumour size and allow a better control by surgery (second-look surgery) or reduced-volume irradiation. Sequelae of surgery or radiation can sometimes appear later and worsen with time. This has a negative impact on the patients and their parents who already carry the burden of a potentially life-threatening disease. Early rehabilitation programmes and psychological support to the patients and their families are a fundamental pre-requisite for the long-term care of these children.