Managing teenage/young adult (TYA) brain tumors: a UK perspective.

Tumors of the CNS are among the commonest malignancies occurring in teenage/young adult patients (i.e., those aged between 15 and 24 years). The treatment of this patient population is challenging. Adolescence and young adulthood are a turbulent period of life, with physical, emotional, social and cognitive changes. Best practice advocates their treatment in dedicated teenage/young adult units, with multidisciplinary team input and access to clinical trials. Treatment of CNS malignancies is dependent upon histological subtype and staging, with varying combinations of surgery, radiotherapy and chemotherapy used. Clinical trials directly targeted at this patient population are rare; treatments are based on pediatric protocols as studies have demonstrated improved outcomes in patients (with other malignancies) treated as such. Scope for improvement lies in minimizing patient risk of recurrence and long-term sequelae of treatment. Molecular characterization of tumors may provide further information.

[Malformation of cortical development: which strategy is best?]. / Malformations du développement cortical: quelles stratégies ?

Malformations of cortical development (MCD) correspond to a broad spectrum of cerebral lesions resulting from cortical development abnormalities during embryogenesis. They are frequently associated with drug-resistant epilepsy as well as more or less severe neurological and cognitive deficits. Diagnosis of MCD has greatly improved with the progress in contemporary imaging techniques, and patients with cryptogenic epilepsy are increasingly recognized as having MCD. Current classifications based on the combination of clinical, imaging, genetic and pathological data allow analysis of homogeneous patient series and optimal therapeutic strategies. Successful surgical treatment can be proposed to patients with focal lesions such as focal cortical dysplasia or tumors associated with cortical dysplasia (dysembryoplastic neuroepithelial tumors and gangliogliomas). Favorable outcome can also be obtained in some diffuse cases such as tuberous sclerosis, periventricular heterotopia and polymicrogyria. Invasive monitoring, especially stereoelectroencephalography (SEEG), has proved to be useful in determining the organization of the epileptogenic zone in each MCD type and planning cortical resections. Moreover, establishing correlations between neurophysiological data, imaging and pathological findings has allowed surgery without previous invasive procedures in the majority of focal MCD types; however, intracranial recordings remain necessary in case of more diffuse MCD.

Practice of palliative sedation in children with brain tumors and sarcomas at the end of life.

Despite progress in the treatment of pediatric cancer, approximately 25% of these children will die of the disease. The last period of life is characterized by profound physical and psychological suffering, both of the children and their loved ones. Adequate alleviation of this suffering becomes the priority in the management of these patients. The authors retrospectively evaluated the indications, incidence, and characteristics of palliative sedation (PS) in 19 children with brain tumors (BT) and 18 with sarcomas (S) at the end of life. Twelve of the 18 S patients received PS, as did 13 of the 19 BT patients. Indications for initiation of PS for those with BT were seizures and/or pain, for those with S were pain and/or respiratory insufficiency. It was concluded that PS may be the only efficacious and safe treatment for the alleviation of suffering in these children at the end of life, despite differing indications.

[Psychosis in a case of temporal lobe epilepsy associated with a dysembryoplastic neuroepithelial tumour]. / Psicosis en un caso de epilepsia temporal asociado a un tumor neuroepitelial disembrioplasico.

INTRODUCTION: Psychosis is an acknowledged, although infrequent, complication that occurs in 0.5 9% of all cases of epilepsy. In this work, we present a case of complex partial epilepsy due to a dysembryoplastic neuroectodermal tumour in the right hippocampus, which began as acute psychosis and was cured following its surgical removal. Both its aetiology and its pathogenesis are discussed. CASE REPORT: We describe the case of a 17 year old male with a family history of schizophrenia. While studying his last years of secondary education, and apparently well adapted, he was taken into custody and hospitalised urgently because of personality disorders. The patient suffered from somatic and auditory hallucinations, simple visual pseudohallucinations, formal alterations to his thinking, cosmovisions, delusions of influence, persecution, grandeur and of a mystic nature, with no other neurological focus. At the same time, the patient also had complex partial seizures. Magnetic resonance imaging revealed a cystic tumour in the right temporal lobe. Pathology laboratory findings showed a dysembryoplastic neuroepithelial tumour. For a period of two years following surgical removal, the schizophrenic symptoms and the complex partial seizures disappeared and the patient no longer required antipsychotic medication. CONCLUSIONS: Epileptic psychosis may be related to pathological conditions of the hippocampus. In this study we present what we believe to be the first case of a dysembryoplastic neuroepithelial tumour giving rise to such psychosis. Surgical removal of the tumour cured both entities.