Very long-term outcomes of pediatric patients treated for optic pathway gliomas: A longitudinal cohort study.

BACKGROUND: Optic pathway gliomas (OPG) represent 5% of childhood brain tumors. Successive relapses lead to multiple treatments exposing to late complications. METHODS: We included patients treated at Gustave Roussy (GR) between 01.1980 and 12.2015 for OPG, before 18 years-old and alive at 5 years from diagnosis. Mortality and physical health conditions data were extracted from medical data files and updated thanks to the GR long-term follow-up program and French national mortality registry for patients included in the French Childhood Cancer Survivor Study. RESULTS: We included 182 5y-OPG-childhood survivors in the analysis (sex-ratio M/F 0.8, 35% with NF1). With a median follow-up of 17.2y (range=5-41), we registered 82 relapses, 9 second malignancies and 15 deaths as first events after 5 years, resulting in 20-y conditional overall survival (C-OS) and late events-free survival (LEFS) of 79.9% (95%CI=71-86) and 43.5% (95%CI=36-51) respectively. NF1 (Hazard ratio HR=3, 95%CI=1.4-6.8), hypothalamic involvement (HR=3.2, 95%CI=1.4-7.3), and radiotherapy (HR=2.8, 95%CI=1.1-6.7) were significantly associated with C-OS in multivariable analyses. Ninety-five percent of 5y-OPG survivors suffered from any health condition, especially visual acuity "<1/10" (n=109), pituitary deficiency (n=106) and neurocognitive impairment (n=89). NF1 (HR 2.1) was associated with precocious puberty. With a median time post diagnosis of 4.2 years, 33 cerebrovascular events were observed in 21 patients. CONCLUSION: Late relapses, second malignancies and cerebrovascular diseases are severe late events resulting in premature mortality. Morbidity is high and needing after-cancer care to improve quality of life. Risk factors could be considered to better stratify long-term follow-up.

Exclusive Hyperfractionated Radiation Therapy and Reduced Boost Volume for Standard-Risk Medulloblastoma: Pooled Analysis of the 2 French Multicentric Studies MSFOP98 and MSFOP 2007 and Correlation With Molecular Subgroups.

PURPOSE: Medulloblastoma has recently been characterized as a heterogeneous disease with 4 distinct molecular subgroups: wingless (WNT), sonic hedgehog (SHH), group 3, and group 4, with a new definition of risk stratification. We report progression-free survival, overall survival, and long-term cognitive effects in children with standard-risk medulloblastoma exclusively treated with hyperfractionated radiation therapy (HFRT), reduced boost volume, and online quality control, and we explore the prognostic value of biological characteristics in this chemotherapy-naïve population. METHODS AND MATERIALS: Patients with standard-risk medulloblastoma were enrolled in 2 successive prospective multicentric studies, MSFOP 98 and MSFOP 2007, and received exclusive HFRT (36 Gy, 1 Gy/fraction twice daily) to the craniospinal axis followed by a boost at 68 Gy restricted to the tumor bed (1.5 cm margin), with online quality assurance before treatment. Patients with MYC or MYCN amplification were not excluded at the time of the study. We report progression-free survival and overall survival in the global population, and according to molecular subgroups as per World Health Organization 2016 molecular classification, and we present cognitive evaluations based on the Wechsler scale. RESULTS: Data from 114 patients included in the MSFOP 98 trial from December 1998 to October 2001 (n = 48) and in the MSFOP 2007 from October 2008 to July 2013 (n = 66) were analyzed. With a median follow-up of 16.2 (range, 6.4-19.6) years for the MSFOP 98 cohort and 6.5 (1.6-9.6) years for the MSFOP 2007 cohort, 5-year overall survival and progression-free survival in the global population were 84% (74%-89%) and 74% (65%-81%), respectively. Molecular classification was determined for 91 patients (WNT [n = 19], SHH [n = 12], and non-WNT/non-SHH [n = 60]-including group 3 [n = 9], group 4 [n = 29], and not specified [n = 22]). Our results showed more favorable outcome for the WNT-activated subgroup and a worse prognosis for SHH-activated patients. Three patients had isolated extra-central nervous system relapse. The slope of neurocognitive decline in the global population was shallower than that observed in patients with a normofractionated regimen combined with chemotherapy. CONCLUSIONS: HFRT led to a 5-year survival rate similar to other treatments combined with chemotherapy, with a reduced treatment duration of only 6 weeks. We confirm the MSFOP 98 results and the prognostic value of molecular status in patients with medulloblastoma, even in the absence of chemotherapy. Intelligence quotient was more preserved in children with medulloblastoma who received exclusive HFRT and reduced local boost, and intelligence quotient decline was delayed compared with patients receiving standard regimen. HFRT may be appropriate for patients who do not consent to or are not eligible for prospective clinical trials; for patients from developing countries for whom aplasia or ileus may be difficult to manage in a context of high cost/effectiveness constraints; and for whom shortened duration of RT may be easier to implement.

Intellectual, educational, and situation-based social outcome in adult survivors of childhood medulloblastoma.

PURPOSE: To investigate intellectual and situation-based social outcome and educational achievement in adult survivors of childhood medulloblastoma and analyse factors influencing outcome Methods: We collected demographic, medical and cognitive data, and social and educational outcome at a mean time since the end of treatments of 14.9 years in 58 adults, aged 19-35 years, consecutively treated in a single cancer center between 1989 and 2005. RESULTS: Ten survivors had severe intellectual disability, 12 were still studying, 23 had a regular employment and 13 were unemployed. Full Scale Intellectual Quotient, assessed 6.6 years after the end of treatments, ranged from 46 to 131. It was strongly associated with educational achievement and significantly lower in patients who experienced postoperative cerebellar mutism, and when parental education level was low. CONCLUSION: These factors should be systematically considered at diagnosis in order to offer adequate and timely assessments and interventions.

Cognitive Profile of Children With Intracranial Germ Cell Tumor According to Tumor Location.

PURPOSE: The purpose of this study was to evaluate the long-term cognitive sequelae and to describe the neuropsychological profile of patients with intracranial germ cell tumors according to tumor location (pineal or suprasellar site). PATIENTS AND METHODS: Forty-five children treated at Gustave Roussy between 1991 and 2010 were assessed with neuropsychological tests to measure IQ, memory, visuospatial, motor, and executive skills at a mean delay of 4.2 years after diagnosis. All patients have received chemotherapy associated with surgery in 17 cases. Thirty-nine patients received, radiotherapy (focal 27, focal plus ventricles 8, craniospinal 4). Twenty-three patients had 2 IQ assessments with a mean delay of 4.1 years between the first and second. RESULTS: Full scale IQ was preserved, with higher verbal IQ than other IQ indexes. Visuospatial, fine-motor, and executive difficulties were present in a significant proportion of patients. Visuospatial and fine-motor deficits were significantly associated with oculomotor difficulties, more present in the pineal than in the suprasellar group. No cognitive decline was observed between the first and the second IQ assessment. CONCLUSIONS: Overall cognitive abilities were preserved in children treated for central nervous system germ cell tumor.

Time knowledge difficulties following treatment for malignant cerebellar tumors.

In children treated for malignant cerebellar tumors, there are only a few studies investigating temporal skills, despite the role of the cerebellum in time processing being generally acknowledged. Children's time knowledge has been defined as the correct representation and use of familiar time units. The present study compares time knowledge in 38 children treated for malignant cerebellar tumors (mean age 11.6 years) with 105 typically-developing (TD) children. The performances on all time knowledge subtests were significantly lower in the tumor group. The results also confirm a lower mean IQ in the children treated for cerebellar tumors, related to slower processing speed and poorer performance on working memory and non-verbal tasks. However, the lower IQ does not explain the considerable difficulties in the acquisition of time knowledge. These results are discussed in the light of the role of the cerebellum in time processing and in the context of two different models of temporal processing: the internal clock model and the neural network state model.

Relationships between Regional Radiation Doses and Cognitive Decline in Children Treated with Cranio-Spinal Irradiation for Posterior Fossa Tumors.

Pediatric posterior fossa tumor (PFT) survivors who have been treated with cranial radiation therapy often suffer from cognitive impairments that might relate to IQ decline. Radiotherapy (RT) distinctly affects brain regions involved in different cognitive functions. However, the relative contribution of regional irradiation to the different cognitive impairments still remains unclear. We investigated the relationships between the changes in different cognitive scores and radiation dose distribution in 30 children treated for a PFT. Our exploratory analysis was based on a principal component analysis (PCA) and an ordinary least square regression approach. The use of a PCA was an innovative way to cluster correlated irradiated regions due to similar radiation therapy protocols across patients. Our results suggest an association between working memory decline and a high dose (equivalent uniform dose, EUD) delivered to the orbitofrontal regions, whereas the decline of processing speed seemed more related to EUD in the temporal lobes and posterior fossa. To identify regional effects of RT on cognitive functions may help to propose a rehabilitation program adapted to the risk of cognitive impairment.

Quality of survival and cognitive performance in children treated for medulloblastoma in the PNET 4 randomized controlled trial.

BACKGROUND: The relationship between direct assessments of cognitive performance and questionnaires assessing quality of survival (QoS) is reported to be weak-to-nonexistent. Conversely, the associations between questionnaires evaluating distinct domains of QoS tend to be strong. This pattern remains understudied. METHODS: In the HIT-SIOP PNET4 randomized controlled trial, cognitive assessments, including Full Scale, Verbal and Performance IQ, Working Memory, and Processing Speed, were undertaken in 137 survivors of standard-risk medulloblastoma from 4 European countries. QoS questionnaires, including self-reports and/or parent reports of the Behavior Rating Inventory of Executive Function (BRIEF), the Health Utilities Index, the Strengths and Difficulties Questionnaire, and the Pediatric Quality of Life Inventory, were completed for 151 survivors. Correlations among direct cognitive assessments, QoS questionnaires, and clinical data were examined in participants with both assessments available (n = 86). RESULTS: Correlations between direct measures of cognitive performance and QoS questionnaires were weak, except for moderate correlations between the BRIEF Metacognition Index (parent report) and working memory (r = .32) and between health status (self-report) and cognitive outcomes (r = .35-.44). Correlations among QoS questionnaires were moderate to strong both for parent and self-report (r = .39-.76). Principal Component Analysis demonstrated that questionnaires and cognitive assessments loaded on 2 separate factors. CONCLUSIONS: We hypothesize that the strong correlations among QoS questionnaires is partially attributable to the positive/negative polarity of all questions on the questionnaires, coupled with the relative absence of disease-specific questions. These factors may be influenced by respondents' personality and emotional characteristics, unlike direct assessments of cognitive functioning, and should be taken into account in clinical trials.

Describing the attention profile of children and adolescents with acquired brain injury using the Virtual Classroom.

OBJECTIVES: The objectives of the study were: (1) to describe the attention deficits profile of children with significant acquired brain injury (ABI) in comparison to matched controls, using the virtual classroom (VC); (2) to assess the utility of the VC in detecting attention deficits in children with ABI, as compared to classical neuropsychological tests and questionnaire-based assessment of attention; and (3) to determine how performance in the VC is affected by demographic and injury severity variables. METHODS: Forty-one children with ABI and 35 age- and gender-matched controls, aged 8-16, were assessed with the VC. The results of the VC were compared to sub-tests of the Test of Everyday Attention for Children (TEA-Ch), the Conners' Parent Rating Scales-Revised: Short (CPRS-R:S) questionnaire and analysed according to demographic and injury severity variables. RESULTS: Significant differences were found between the groups regarding the number of targets correctly identified in the VC. Significant inter-correlations were obtained between the VC variables. Significant correlations were found between the VC variables, the sub-tests of TEA-Ch and the CPRS-R:S and the demographic characteristics of the sample. CONCLUSION: The VC appears to be a sensitive and ecologically valid assessment tool for use in the diagnosis of attention deficits among children with ABI.

Neuropsychological Outcome of Children Treated for Standard Risk Medulloblastoma in the PNET4 European Randomized Controlled Trial of Hyperfractionated Versus Standard Radiation Therapy and Maintenance Chemotherapy.

PURPOSE: In the European HIT-SIOP PNET4 randomized controlled trial, children with standard risk medulloblastoma were allocated to hyperfractionated radiation therapy (HFRT arm, including a partially focused boost) or standard radiation therapy (STRT arm), followed, in both arms, by maintenance chemotherapy. Event-free survival was similar in both arms. Previous work showed that the HFRT arm was associated with worse growth and better questionnaire-based executive function, especially in children <8 years of age at diagnosis. Therefore, the aim of this study was to compare performance-based cognitive outcomes between treatment arms. METHODS AND MATERIALS: Neuropsychological data were collected prospectively in 137 patients. Using the Wechsler Intelligence Scales, Kaufman Assessment Battery for Children, and Raven's Progressive Matrices, we estimated full-scale intelligence quotient (FSIQ) and, when available, verbal IQ (VIQ), performance IQ (PIQ), working memory index (WMI), and processing speed index (PSI). RESULTS: Among the 137 participants (HFRT arm n=71, STRT arm n=66, 63.5% males), mean (±SD) ages at diagnosis and assessment respectively were 9.3 (±3.2) years of age (40.8% < 8 years of age at diagnosis) and 14.6 (±4.3) years of age. Mean (±SD) FSIQ was 88 (±19), and mean intergroup difference was 3.88 (95% confidence interval: -2.66 to 10.42, P=.24). No significant differences were found in children >8 years of age at diagnosis. In children <8 years of age at diagnosis, a marginally significant trend toward higher VIQ was found in those treated in the HFRT arm; a similar trend was found for PSI but not for PIQ, WMI, or FSIQ (mean intergroup differences were: 12.02 for VIQ [95% CI: 2.37-21.67; P=.02]; 3.77 for PIQ [95% CI: -5.19 to 12.74; P>.10]; 5.20 for WMI [95% CI: -2.07 to 12.47; P>.10]; 10.90 for PSI [95% CI: -1.54 to 23.36; P=.08]; and 5.28 for FSIQ [95% CI: -4.23 to 14.79; P>.10]). CONCLUSIONS: HFRT was associated with marginally higher VIQ in children <8 years of age at diagnosis, consistent with a previous report using questionnaire-based data. However, overall cognitive ability was not significantly different.

[Neurocognitive outcome in childhood brain tumor survivors]. / Devenir neurocognitif à long terme des enfants traités pour une tumeur cérébrale.

Advances in treatment have significantly improved the survival rate of children with brain tumour. This review describes risk factors of late neurocognitive effect, the different type of neurocognitive side effect according to the type of tumour and the rehabilitation. The best knowledge of cognitive sequelae allowed to propose supported appropriate and specific disorders including school facilities for younger and adaptation of professional situation for adults. Furthermore, this understanding allows to link with the patient's quality of life and to approach the person in its specificity and overall.

Conceptions of time in children treated for malignant cerebellar tumours.

PRIMARY OBJECTIVE: Conceptions of time in children having survived malignant cerebellar tumours (CT) and healthy children matched for chronological age (HCCA) were compared, knowing that the cerebellum has been involved in time perception. METHODS AND PROCEDURE: Study participants included 20 children with CT (13 boys) and 20 HCCA (10 boys) aged 6-12 years. All children with CT were at least 1 year after the end of treatment without relapse. A time questionnaire (TQ) exploring duration of daily activities, time units, planning and diachronic thinking was used, as well as a video animation (VA) displaying cyclic and linear time. MAIN OUTCOMES AND RESULTS: Children with CT obtained similar results as HCCA for time units, planning and diachronic thinking, but showed more difficulties than controls in estimating the duration of daily activities and understanding linear and cyclic time concepts (VA). CONCLUSIONS: These findings are not in favour of impaired time conceptions in children with CT and are probably linked to the specific life experience among children treated for a malignant pathology, rather than to the role of the cerebellum in time processing.

Disrupted sensorimotor synchronization, but intact rhythm discrimination, in children treated for a cerebellar medulloblastoma.

The aim of this study was to investigate the temporal abilities of children treated by surgery for a malignant tumor in the cerebellum, both in the perception and the production of rhythm. Children with a diagnosed medulloblastoma and age-matched control children were tested in a rhythm discrimination task and a sensorimotor synchronization task. Their motor and cognitive capabilities were also assessed through a battery of age-adapted neuropsychological tests. The results did not show any significant difference in performance between groups for the discrimination task. On the contrary, children with cerebellar lesions produced longer and more variable inter-tap intervals (ITI) in their spontaneous motor tempo (SMT) than did the control children. However, the length and, to a lesser extent, the variability of their SMT decreased after a synchronization phase, when they had been instructed to tap in synchrony with a beep. During the synchronization task, the children with medulloblastoma succeeded to modify the length of their ITI in response to an auditory rhythm, although with better success when the inter-stimuli intervals (ISI) were shorter than when they were longer than the ITIs of their own SMT. Correlational analyses revealed that children's poorer synchronization performance was related to lower scores in neuropsychological tests assessing motor dexterity and processing speed.

Tandem high-dose chemotherapy and autologous stem cell rescue in children with newly diagnosed high-risk medulloblastoma or supratentorial primitive neuro-ectodermic tumors.

BACKGROUND: To assess the feasibility and effectiveness of high-dose chemotherapy (HDC) with stem cell support followed by conventional craniospinal radiotherapy (RT) as treatment for children older than 5 years of age with newly diagnosed high-risk medulloblastoma (MB) or supratentorial PNET (sPNET). PROCEDURE: Between May 2001 and April 2010, 24 children older than 5 years of age (MB = 21; sPNET = 3), fulfilling inclusion criteria at diagnosis, were treated at Gustave Roussy. After conventional chemotherapy, they received two courses of high-dose thiotepa (600 mg/m(2)) followed by craniospinal RT. RESULTS: The median follow-up was 4.4 years (range, 0.8-11.3 years). For children with metastatic MB, the 5-year event-free survival (EFS) and overall survival (OS) were 72% and 83%, respectively. The toxicity was manageable. No toxic death occurred. At the most recent evaluation, among the 24 children who had at least one Full Scale Intellectual Quotient (FSIQ) examination at a median follow-up of 3.79 years after diagnosis, the mean estimated FSIQ was 82 (range, 56-114). CONCLUSIONS: In children with metastatic MB, tandem HDCT with ASCT followed by conventional craniospinal RT proved its feasibility without jeopardizing survival.

High-dose busulfan-thiotepa with autologous stem cell transplantation followed by posterior fossa irradiation in young children with classical or incompletely resected medulloblastoma.

BACKGROUND: The aim of the study is to evaluate the outcome of young children with high risk localized medulloblastomas (newly diagnosed classical or incompletely resected) treated by high-dose busulfan-thiotepa with autologous stem cell rescue (ASCT) followed by focal radiation therapy (RT). PROCEDURE: Between September 1994 and January 2010, 19 children younger than 5 years old at diagnosis fulfilling the above inclusion criteria were treated at the Institute Gustave Roussy. After conventional chemotherapy, they received busulfan at a dose of 600 mg/m(2) and thiotepa at a dose of 900 mg/m(2) followed by ASCT. Focal RT was delivered at least 70 days after ASCT. RESULTS: The median follow-up was 40.5 months (range, 14.5-191.2 months). The 3-year event-free survival (EFS) and OS were 68% (95% CI 45-84%) and 84% (95% CI 61-94%), respectively. Acute toxicity consisted mainly in hepatic veno-occlusive disease (6/19 patients) and bone marrow aplasia (all patients). No toxic death occurred. The Full Scale Intellectual Quotient tended to decrease over time at a mean rate of 0.9 point per year from the date of diagnosis. CONCLUSIONS: This intensive treatment resulted in a high overall survival rate in young children with newly diagnosed non-metastatic classic or incompletely resected MB. In spite of a high incidence of hepatic veno-occlusive disease (32%), the acute toxicity was manageable. Delayed neuropsychological side effects remain main concerns. These results should to be confirmed in a larger cohort.

Time perception in children treated for a cerebellar medulloblastoma.

The aim of the present study was to investigate temporal abilities in children treated by surgery for a malignant tumor in the cerebellum. Children with a diagnosed medulloblastoma and age-paired control children were given a temporal discrimination task (bisection task) and a temporal reproduction task with two duration ranges, one shorter than 1s and the other longer than 4s. The motor and cognitive capacities of these children were also assessed by a battery of age-adapted neuropsychological tests. The results did not show any significant difference in performance between the children with or without cerebellar lesions in the temporal discrimination task. It was only in the temporal reproduction task that the children with cerebellar lesions reproduced longer and more variable durations than the other children, but only for the short stimulus durations (≤ 1 s). In addition, a hierarchical regression analysis revealed that the best predictor of variance in temporal performance was a significantly lower processing speed in children with cerebellar lesions in comparison to their controls. These results indicated that the major cause of deficits in temporal judgments in children with cerebellar lesions was due to their inability to reproduce accurately short temporal intervals in association with low processing speed, rather than to a specific deficit in the perception of time.

Pseudoprogression after high-dose busulfan-thiotepa with autologous stem cell transplantation and radiation therapy in children with brain tumors: Impact on survival.

Children with a brain tumor treated with high-dose busulfan-thiotepa with autologous stem cell transplantation (ASCT) and radiation therapy (RT) often experience radiographic changes during follow-up. The purpose of the study was to identify the incidence, time course, risk factors, and clinical outcome of this complication. From May 1988 through May 2007, 110 patients (median age, 3.6 years; range, 1 month to 15.3 years) with a brain tumor had received 1 course of high-dose busulfan-thiotepa with stem cell rescue, followed or preceded by RT as part of their treatment. All MRI follow-up examinations were systematically reviewed. Twenty-three patients (21%) developed neuroradiological abnormalities at a median time of 9.2 months (range, 5.6-17.3 months) after ASCT. All contrast-enhancing lesions appeared in patients who had received RT after ASCT and were localized inside the 50-55Gy isodoses. They disappeared in 14 of 23 patients after a median time of 8 months (range, 3-17 months), leaving microcalcifications in some cases. The presence of MRI abnormalities was an independent prognostic factor for overall survival in the multivariate analysis (hazard ratio, 0.12; 95% confidence interval [CI], 0.04-0.33), with a 5-year overall survival rate of 84% among patients with MRI abnormalities (95% CI, 62-94), compared with 27% (95% CI, 19-37) among those without lesions. MRI-detectable pseudoprogression is a common early finding in children treated with high-dose busulfan-thiotepa followed by radiation therapy and is correlated with a better outcome.

Teachers' report of learning and behavioural difficulties in children treated for cerebellar tumours.

PRIMARY OBJECTIVE: The aim of the study was the validation of the Deasy-Spinetta Questionnaire (DSQ) in 6-11 year olds with attention to the verification of three factors (learning difficulties, socialization and emotionality) and its application in children treated for cerebellar tumour. METHODS AND PROCEDURES: Twenty-nine children aged between 6-11 years were compared with 609 classmates. Teachers completed the DSQ. Patients were evaluated according to Wechsler Scales, the Purdue Pegboard and the International Cooperative Ataxia Rating scale. MAIN OUTCOMES AND RESULTS: In classmates, the DSQ factorial analysis showed three factors: learning, socialization difficulties and disturbing behaviour. Teachers reported more learning difficulties in patients than in classmates. Learning difficulties in patients were greater for mathematics and reasoning than for reading. Patients were described as less active, without evidence of autistic-like behaviour or irritability. The teachers' report of learning difficulties was significantly correlated with IQ scores, but not with neurological deficits. CONCLUSIONS: The proposed DSQ scores are interesting for the assessment of learning and behavioural difficulties in children treated for cerebellar tumours, as they provide complementary ecological information to that given by clinical and neuropsychological testing.

Isolated corpus callosum agenesis: a ten-year follow-up after prenatal diagnosis (how are the children without corpus callosum at 10 years of age?).

BACKGROUND: Corpus callosum agenesis (CCA) is generally diagnosed in utero. Outcome appears to be better if the malformation is isolated. The aim of this study, which is the first one with a long (10 years) and standardized follow up, was to report cognitive abilities of children with isolated CCA diagnosed prenatally. METHODS: We prospectively evaluated 17 children. Clinical examinations, neuropsychological tests were performed each year. School achievement and personal and familial data were collected. RESULTS: Twelve children completed the entire follow up. One child was finally considered to have associated CCA, because signs of fetal alcohol syndrome had become obvious. Of the 11 other children, three (27%) had borderline intelligence whereas the intelligence levels of eight (73%) were in the normal range, although half of these children experienced some difficulties in scholastic achievement. Neither epilepsy nor intellectual deficiency was noted and intellectual quotient scores correlated strongly with the mother's education level. CONCLUSION: Although prenatal diagnosis of isolated CCA is reliable, false postnatal diagnoses remain possible (10-20%) even with complete prenatal screening. Outcome is mostly favorable because intelligence is within the normal range for nearly 3/4 of the children. However, they frequently have mild learning difficulties.

Cognitive and academic outcome after benign or malignant cerebellar tumor in children.

OBJECTIVE: To examine the impact of malignancy and location of the cerebellar tumor on motor, cognitive, and psychologic outcome. BACKGROUND: Although many studies focus on long-term outcome after cerebellar tumor treatment in childhood, the impact of its precise location remains unclear. PATIENTS AND METHODS: Children, aged from 6 to 13 years, with a cerebellar malignant tumor (MT; MT group, n=20) or a cerebellar benign tumor (BT; BT group, n=19) were examined at least 6 months after the end of treatment using the international cooperative ataxia rating scale, the Purdue pegboard for manual skill assessment and the age-adapted Weschler scale. Structural changes in brain anatomy were evaluated and parents and teachers answered 2 independent questionnaires. RESULTS: Parents and teachers reported high rate of learning and academic difficulties, but without any difference with respect to the type of tumor. However, children with cerebellar MT showed increased cognitive and motor difficulties compared with children with cerebellar BT. Cerebellar signs at clinical examination and manual skill impairment were strongly associated with cognitive difficulties. Both motor and cognitive impairments were found to be associated with extension of the lesion to the dentate nuclei. CONCLUSIONS: Dentate nuclei lesions are major risk factors of motor and cognitive impairments in both cerebellar BT and MT.

Forniceal glioma in children. Clinical article.

OBJECT: Five to ten percent of pediatric brain tumors are located in the ventricles. Among them, forniceal lesions are rare and their management has not often been described. The aim of this study was to review the clinical, radiological, and histopathological features as well as the feasibility of surgical excision and the outcomes in these patients. METHODS: From a retrospective analysis of 250 cases of supratentorial pediatric glioma, the records of 8 children presenting with forniceal lesions were selected and reviewed. RESULTS: The median age of patients in the cohort was 13.5 years. Presenting features included intracranial hypertension (7 cases), hypothalamic dysfunction (2), and memory dysfunction (3). Complete resection was possible in only 1 case, where the lesion was mainly exophytic; the remaining patients had either a partial resection or biopsy. On histological review, the tumors were confirmed as pilocytic astrocytoma (4 lesions), WHO Grade II astrocytoma (3), and ganglioglioma (1). Postoperatively, working and retrograde memory was normal for all patients, but the authors found a mild alteration in verbal episodic memory in 5 patients. Despite fatigability for 5 patients, academic achievement was normal for all but 2, both of whom had preoperative school difficulties. Additional treatment was required for 5 patients for tumor progression, with a median interval of 19 months from surgery. At a median follow-up duration of 4.9 years, all patients had stable disease. CONCLUSIONS: In this series, forniceal gliomas were found to be low-grade gliomas. They are surgically challenging, and only exophytic lesions may be cured surgically. Due to the high rate of progression of residual disease, adjuvant therapy is recommended for infiltrative tumors, and it yielded excellent results.