paediatrics Brussels 17

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7. Late sequelae

treatment should be installed before irreversible deficits has developed.

7.1. Long term sequelae

Cognitive and focal neurological deficits may have a great impact on long term survivors of brain tumours, regardless of the histology and grade of the tumour. Memory loss, apa- thy, concentration difficulties and personality changes may have a profound effect, even in those patients who appear to have a Karnofsky performance status of 90 or 100. Surgery in the so-called silent areas may contribute to cognitive deficits. Less clear are the late effects of radiation therapy on cog- nitive function. Radiotherapy is known to cause the early somnolence syndrome, but may also cause late sequelae, in particular delayed leuko-encephalopathy with cognitive dysfunction and radiation necrosis [95–97] . In individual patients, it is difficult however to entangle the direct effects of the tumour on cognition from late effects of treatment. A recent survey on cognitive deficits in progression free sur- vivors of low-grade glioma failed to confirm the generally assumed untoward relation between radiotherapy and cogni- tive deficits [98] . Only in those patients who had been treated with fraction of 2 Gy or more, evidence of increased cognitive dysfunction has been observed. The only other associa- tion with cognitive deficits was treatment with anti-epileptic drugs. Prior studies have suggested that whole brain radio- therapy may be associated with more cognitive deficits than involved field irradiation, although today involved field radio- therapy is standard practice [99] . Radiation therapy may also affect cranial nerves, or induce endocrine dysfunction even in case of tumours distant from the hypothalamus-pituitary region [100] . Seizures may have a great impact on the quality of life even in patients with well-controlled tumours. Newer anti-epileptic drugs may have less side-effects and should be considered, especially in those patients using a multi-drug regimen. Apart from cognitive deficits, a risk of death of 2.5% at 2 years has been reported for doses of 50.4 Gy. A risk of radionecrosis up to 5% at 5 years may occur after 60 Gy to one third, or 50 Gy to two thirds of the brain volume, and with 50–53 Gy to brain stem with a similar risk for blindness after 50 Gy to the optic chiasm. Also chemotherapy may induce late sequelae such as lymphoma, leukemia or solid tumours, as well as lung fibrosis, infertility, renal failure, and signs of neurotoxicity of the peripheral nervous system.

Acknowledgment

The authors thank the members of the EUROCAREWork- ing Group for permitting the relative survival analysis from the EUROCARE dataset.

References

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8. Follow-up

8.1. Follow up

No general guidelines for the follow-up of ependymomas can be given, these should be tailored to the individual patient and taking into account tumour grade, previously adminis- tered treatment and independent prognostic factors as age and the functional status of the patient. Low-grade glioma patients should be followed, even with stable lesion since many years. At some point in time, progression will inevitably occur and