Role of neoadjuvant chemotherapy in metastatic medulloblastoma: a comparative study in 92 children.

BACKGROUND: Previous pilot studies have shown the feasibility of preoperative chemotherapy in patients with medulloblastoma, but benefits and risks compared with initial surgery have not been assessed. METHODS: Two therapeutic strategies were retrospectively compared in 92 patients with metastatic medulloblastoma treated at Gustave Roussy between 2002 and 2015: surgery at diagnosis (n = 54, group A) and surgery delayed after carboplatin and etoposide-based neoadjuvant therapy (n = 38, group B). Treatment strategies were similar in both groups. RESULTS: The rate of complete tumor excision was significantly higher in group B than in group A (93.3% vs 57.4%, P = 0.0013). Postoperative complications, chemotherapy-associated side effects, and local progressions were not increased in group B. Neoadjuvant chemotherapy led to a decrease in the primary tumor size in all patients; meanwhile 4/38 patients experienced a distant progression. The histological review of 19 matched tumor pairs (before and after chemotherapy) showed that proliferation was reduced and histological diagnosis feasible and accurate even after neoadjuvant chemotherapy. The 5-year progression-free and overall survival rates were comparable between groups. Comparison of the longitudinal neuropsychological data showed that intellectual outcome tended to be better in group B (the mean predicted intellectual quotient value was 6 points higher throughout the follow-up). CONCLUSION: Preoperative chemotherapy is a safe and efficient strategy for metastatic medulloblastoma. It increases the rate of complete tumor excision and may improve the neuropsychological outcome without jeopardizing survival. KEY POINTS: 1. Preoperative chemotherapy increases the rate of complete tumor removal.2. No additional risk (toxic or disease progression) is linked to the delayed surgery.3. Preoperative chemotherapy could have a positive impact on the neuropsychological outcome of patients.

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.

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.