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Background Little is known about cognition and predictors of neuropsychological outcomes in pediatric low-grade glioma (PLGG) survivors treated without radiation therapy. This research expands upon our previous work by further identifying the cognitive profile of PLGG patients treated without radiation therapy, investigating the specific medical and demographic variables that predict functioning, and examining white matter structure and its relationship to neuropsychological performance. Procedure Nineteen PLGG patients (11-19 years) were administered the Wechsler Intelligence Scale for Children/Wechsler Adult Intelligence Scale, and subtests from the Woodcock-Johnson Tests of Cognition (visual matching, rapid picture naming, and pair cancellation) and Cambridge Neuropsychological Test Automated Battery (pattern recognition memory, delayed matching to sample, intra-extra dimensional set shift, motor screening task, rapid visual information processing, and spatial span). Results The sample had normative weaknesses in verbal working memory, brief attention/vigilance, psychomotor speeded output, visual perception and matching, overall cognition, working memory, and processing speed. Increased surgeries or subtotal resections, hydrocephalus, shunting procedures, chemotherapy, NF1, and supratentorial location were predictive of cognitive deficits. Broad white matter involvement of the frontal, temporal, parietal, and occipital lobes as well as the cerebellum, as inferred from diffusion tensor imaging indices of decreased fiber orientation and increased water diffusion, was related to many cognitive difficulties. Conclusions This study comprehensively examines cognitive functioning in PLGG patients treated without radiation therapy, predictors of cognition, and its relation to white matter structure. Our findings indicate that medical and demographic variables other than radiation therapy can lead to cognitive late effects with diffuse white matter involvement.

Background Because atypical teratoid rhabdoid tumor(ATRT) is a rare disease of infancy carrying a grim prognosis, focus on long-term outcome, especially neurocognitive remained very limited. With new era of multimodality therapy, an increasing proportion of patients are now long-term survivors. Procedure Retrospective review of neuropsychological (NP) status of survivors from the Canadian ATRT registry. Results Among 77 patients diagnosed between 1995–2012, 16(22%) were survivors. Formal NP assessments were available in eight patients. Partial information on academic achievement was available on three additional patients. There were four girls and seven boys diagnosed at a median age of 27.5 months. Seven patients underwent complete resection and three had metastatic disease. All but one received sequential high dose chemotherapy. Five patients received intrathecal chemotherapy. Three patients underwent radiation. Median age at time of formal NP assessment was 7.6 years (3.9–9.8). Full Scale Intellectual Quotient (FSIQ) ranged from less than 50–119 (mean 78). Simple expressive and receptive language appeared relatively preserved. Three recently diagnosed patients (median time assessment from diagnosis 2.6 years (1.6–4.7)) had average to high average FSIQ, academic and visual spatial skills, visual, and verbal memory. Five other patients diagnosed earlier and tested at a median time of 4.9 years (3.3–8.3) post-diagnosis had a FSIQ ranging from

Background Treatment intensity for pediatric medulloblastoma may vary depending on the type of medulloblastoma. In some cases, the dose of radiation may be reduced or eliminated. Correspondingly, there may be trade-offs between quality of life and survival. In this study, focus groups were conducted with parents and clinicians to explore their opinions about these trade-offs as well as the alignment/misalignment between parents and clinicians regarding the trade-offs. Methods One hour semi-structured focus groups were conducted with parents of children with medulloblastoma and health care providers who were involved in the care of these children. Results Parents and providers showed differences in which factors they believe have the greatest impact on quality of life for children with medulloblastoma and their families. For parents, the most important factor is social functioning and their child's ability to make friends and have a social life. In contrast, providers thought that parents cared most about their child's cognitive functioning and ability to attend and perform in school. Conclusion Understanding parents' perspectives on quality of life is important in terms of providing support services that target the areas that the parents prioritize. The types of functioning that are most strongly correlated with quality of life from the parents' perspective may be the ones that should be targeted to protect during treatment. Pediatr Blood Cancer 2014;61:1300–1304. © 2014 Wiley Periodicals, Inc.

Background The optimal management of optic pathway gliomas (OPGs) is complicated by their variable natural history, the association with neurofibromatosis type 1 (NF1) and difficulties in defining progression and response to treatment. Methods This study is a retrospective review of all children presenting to a single institution with an OPG between 1990 and 2004. Results Of the 133 children included, 78 (59%) had NF1; 87 (71 NF1) were observed initially, of whom 23 (11 NF1) subsequently required treatment. Forty-six patients received immediate treatment. Initial treatment, without or with an observation period, comprised chemotherapy alone (32, 11 NF1); debulking + chemotherapy (15, 4 NF1); gross total resection (6); radiotherapy (2); debulking + radiotherapy (3); and debulking only (12, 3 NF1). Overall, 16 patients were irradiated during the study period. Four children died (overall survival at 5 and 10 years was 97.6% and 94.6% for those who required treatment). Progression-free survival (PFS) for the 69 patients who needed treatment was 48%. There was no difference in PFS between chemotherapy versus chemotherapy + debulking or debulking alone. PFS for the NF1 patients who required treatment was similar to that of non-NF1 patients. Mean follow-up time was 9.0 (range 0.6–18.0, median 8.6) years. Conclusions The study confirms the complexity of OPGs and that NF1 is a major determinant of the resultant behavior of the tumor. Pediatr Blood Cancer 2009; 53:1231–1237. © 2009 Wiley-Liss, Inc.