A B S T R A C T PurposeSurgery is curative therapy for pediatric low-grade gliomas (LGGs) in areas of the brain amenable to complete resection. However, LGGs located in areas where complete resection is not possible can threaten both function and life. The purpose of this study was to compare two chemotherapy regimens for LGGs in children younger than age 10 years for whom radiotherapy was felt by the practitioner to pose a high risk of neurodevelopmental injury. Patients and MethodsPreviously untreated children younger than age 10 years with progressive or residual LGGs were eligible. Children were randomly assigned to receive carboplatin and vincristine (CV) or thioguanine, procarbazine, lomustine, and vincristine (TPCV). Children with neurofibromatosis are reported separately. ResultsOf 274 randomly assigned patients who met eligibility requirements, 137 received CV and 137 received TPCV. The 5-year event-free survival (EFS) and overall survival (OS) rates for all eligible patients were 45% Ϯ 3.2% and 86% Ϯ 2.2%, respectively. The 5-year EFS rates were 39% Ϯ 4% for CV and 52% Ϯ 5% for TPCV (stratified log-rank test P ϭ .10; cure model analysis P ϭ .007). On multivariate analysis, factors independently predictive of worse EFS and OS were younger age and tumor size greater than 3 cm 2 . Tumor location in the thalamus was also associated with poor OS. ConclusionThe difference in EFS between the regimens did not reach significance on the basis of the stratified log-rank test. The 5-year EFS was higher for TPCV on the basis of the cure model analysis. Differences in toxicity may influence physician choice of regimens.

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The optimum treatment of nonresectable low-grade gliomas of childhood remains undecided. There has been increased interest in the use of chemotherapy for young children, but little information concerning the long-term efficacy of such treatment. Seventy-eight children with a mean age of 3 years (range 3 months-16 years) who had newly diagnosed, progressive low-grade gliomas were treated with combined carboplatin and vincristine chemotherapy. The patients were followed for a median of 30 months from diagnosis, with 31 patients followed for more than 3 years. Fifty-eight children had diencephalic tumors, 12 had brainstem gliomas, and three had diffuse leptomeningeal gliomas. Forty-four (56%) of 78 patients showed an objective response to treatment. Progression-free survival rates were 75 +/- 6% at 2 years and 68 +/- 7% at 3 years. There was no statistical difference in progression-free survival rates between children with neurofibromatosis Type 1 and those without the disease (2-year, progression-free survival 79 +/- 11% vs. 75 +/- 6%, respectively). The histological subtype of the tumor, its location, and its maximum response to chemotherapy did not have an impact on the duration of disease control. The only significant prognostic factor was age: children 5 years old or younger at the time of treatment had a 3-year progression-free survival rate of 74 +/- 7% compared with a rate of 39 +/- 21% in older children (p < 0.01). Treatment with carboplatin and vincristine is effective, especially in younger children, in controlling newly diagnosed progressive low-grade gliomas.

Adverse outcome in childhood intracranial ependymoma is related to age (3 years or younger), histology (grade 3), and degree of surgical resection (less than GTR). New approaches, particularly for local tumor control in younger patients, are needed to improve survival.

PURPOSE Childhood cancer survivors (CCSs) are at increased risk for poor health-related quality of life (HRQOL) and chronic health conditions -- both of which can be exacerbated by unhealthy lifestyle behaviors. Developing a clearer understanding of the associations between HRQOL, lifestyle behaviors, and medical and demographic variables (e.g., age/developmental stage at time of diagnosis) is an important step toward developing more targeted behavioral interventions for this population. METHOD Cross-sectional questionnaires were completed by 170 CCSs who were diagnosed with leukemia, lymphoma, sarcoma, or a cancer of the central nervous system (CNS) and treated at a comprehensive cancer center between 1992 and 2007. Questionnaires addressed weight status, lifestyle behaviors, aspects of HRQOL, and intervention preferences. RESULTS Adolescent and young adult survivors (AYAs) and survivors of CNS tumors or lymphoma reported significantly (p<.05) poorer HRQOL across multiple domains compared to those diagnosed at an earlier age, survivors of leukemia or sarcoma, and healthy populations. A significant proportion also failed to meet national recommendations for dietary intakes (39–94%) and physical activity (65%). Female survivors reported poorer physical functioning and consumed less dietary fiber and fruits and vegetables than did male survivors. They also expressed the strongest interest in participating in diet and exercise interventions. CONCLUSION Findings support the premise that females, AYAs, and survivors of cancers of the CNS or lymphoma are “at risk” subgroups within the CCS population for poor dietary practices, sedentary behaviors, and poor HRQOL. Future research should focus on developing diet and PA interventions to improve HRQOL that target these groups. IMPLICATIONS FOR SURVIVORS Greater consideration of the role of gender, developmental stage, and the HRQOL challenges facing CCSs may help researchers to develop targeted behavioral interventions for those who stand to benefit the most.

PURPOSE: To assess the long-term neuropsychologic effects experienced by children who have tumors in the cerebellum that are diagnosed and treated during infancy. PATIENTS AND METHODS: Twenty-seven children with posterior fossa tumors diagnosed at less than 36 months of age were assessed prospectively with a comprehensive set of age-appropriate tests. Group means and SDs are reported for assessments conducted at diagnosis (analysis 1) and at the most recent follow-up appointment (analysis 2). Cognitive developmental growth curves were derived from the prospective data (analysis 3) using mixed model regression analyses and controlling for age at diagnosis and socioeconomic status. RESULTS: In the first analysis, eight of 11 infants at diagnosis scored within normal limits on all neuropsychologic domains, except for motor skills, which were impaired. In the second analysis, mean scores at the most recent follow-up of 21 of 27 patients were mostly in the normal range; however, group comparisons between those who had (n = 7) and had not (n = 14) been treated with cranial radiation therapy (CRT) showed that patients in the irradiated (CRT) group scored significantly lower than those in the nonirradiated (No-CRT) group on verbal intelligence quotient (IQ) and in the motor domain. In the third analysis (growth curves of CRT and No-CRT groups), statistically significant differences in slope were found on verbal IQ, performance IQ, perceptual-motor skills, language, and attention/executive skills. Slopes on the fine-motor domain were similar; both groups declined at approximately the same rate. CONCLUSION: Neurocognitive development and outcome of children with cerebellar tumors diagnosed in infancy is very positive among those who were treated with surgery and chemotherapy. Declines in performance across time were minimal, and scores tended to remain within normal limits. By itself, a cerebellar tumor in infancy does not seem to have a significant impact on children. However, those who received CRT as part of their treatment are likely to have neurocognitive and psychosocial deficits that require remediational interventions.

Objective Survivors of childhood cancer are at an increased risk for reduced quality of life (QOL), yet few studies have explored factors associated with improving health-related QOL (HRQOL) in this population. We thus explored the relationship between physical activity (PA) and HRQOL among survivors of childhood cancer. Methods A total of 215 survivors of childhood lymphoma, leukemia, and central nervous system (CNS) cancers completed mailed surveys that elicited information regarding leisure-time PA (LTPA) measured in metabolic equivalents, HRQOL, and diagnostic and demographic factors. Correlations and adjusted regression models were use to explore the relationship between LTPA and HRQOL. Results In the total sample, modest yet significant linear associations were observed between LTPA and overall HRQOL (β = 0.17, p < 0.01) as well as each of the respective subscales (β = 0.11–0.23 and p < 0.05 to < 0.001). Among adolescent survivors of childhood cancer, LTPA was significantly associated with overall HRQOL (β = 0.27), cancer worry (β = 0.36), cognitive function (β = 0.32), body appearance (β = 0.29), and social function (β = 0.27) (all p < 0.05). Among adult survivors of childhood cancer, LTPA was only significantly associated with physical function (β = 0.28, p < 0.001). Conclusions Significant associations exist between LTPA and HRQOL; however, the association was stronger and observed in more domains for adolescent survivors of childhood cancer. More research is needed to determine the antecedents and consequences of physical activity in this population.

Purpose To evaluate tumor response, event-free survival (EFS), overall survival (OS), and toxicity of chemotherapy, children with NF1 and progressive low-grade gliomas (LGG) were enrolled on COG A9952 protocol and treated with carboplatin and vincristine (CV). Patients and Methods Non-NF1 patients were randomized to CV versus thioguanine, procarbazine, CCNU, vincristine (TPCV) on COG A9952. NF1 patients were assigned CV only. NF1 and non-NF1 patients, who were treated with CV, were compared on baseline characteristics, toxicity, tumor response, EFS, and OS. Results A total of 127 eligible patients with NF1 were non-randomly assigned to CV: 42 (33%) NF1 patients had events and 6 (4.7%) died. The 5-year EFS for the CV-NF1 group was 69% ± 4% versus 39% ± 4% for the CV-non-NF1 group (P<0.001). In univariate analysis, NF1 children had significantly higher tumor response rate and superior EFS and OS compared to CV-treated children without NF1. NF1 and non-NF1 patients differed significantly in amount of residual tumor, extent of resection, tumor location, and pathology. After multivariate analysis, NF1 was independently associated with better EFS (p < 0.001), but not with OS. NF1 patients also had decreased risk of grade 3 or 4 toxicities compared to non-NF1 patients. Three second malignant neoplasms (SMNs) occurred in NF1 patients receiving CV (CV-NF1), at a median of 7.8 years (range 7.3 to 9.4 years) after enrollment, but none in the non-NF1 group. Conclusions Children with NF1 tolerated CV well and had tumor response rate and EFS that were superior to children without NF1.