BACKGROUND: Pediatric gliomas are rare and heterogeneous tumors. The Surveillance, Epidemiology, and End Results (SEER) database allows a large‐scale analysis of the clinical characteristics and prognostic features of these tumors.METHODS: The authors analyzed available SEER data on 6212 patients younger than 20 years at diagnosis of glioma (1973‐2005), according to 4 age categories: <1 year, 1‐3 years, 3‐5 years, and 5‐20 years.RESULTS: The overall 5‐ and 10‐year survival estimates were 71% ± 0.62% (standard error) and 68% ± 0.67%, respectively. Forty‐one percent of gliomas were cerebral; the frequency of cerebellar tumors (22%‐32% of gliomas) increased sharply after the first year of life. Of the tumors for which grade was available, 77% were low grade (grade I or II). Tumor grade emerged as the most significant independent prognostic factor in all age groups except the youngest age group, in which extent of resection was most significant. Surgery other than gross total resection was an adverse prognostic factor (hazard ratio, 2.18; 95% confidence interval, 1.78‐2.67). Age <3 years predicted a greater likelihood of survival in patients with high‐grade gliomas and brainstem tumors. Conversely, age <3 years predicted a lower likelihood of survival in patients with low‐grade gliomas. Children aged <1 year received less radiotherapy than older patients (P < .0001) and were less likely to undergo gross total resection (P < .0001).CONCLUSIONS: The survival of children with gliomas is influenced by histologic subtype, age, and extent of resection. Despite its limitations, the SEER database provides a useful tool for studies of rare tumors such as pediatric gliomas. Cancer 2009. © 2009 American Cancer Society.

Purpose: Carboplatin-induced ototoxicity remains poorly defined but is of potential great consequence in children with retinoblastoma. We retrospectively assessed the incidence of ototoxicity and its risk factors in children with retinoblastoma who were treated with carboplatin.Patients and Methods: We reviewed the audiologic test results of 60 patients with retinoblastoma who received front-line treatment with systemic carboplatin and vincristine according to the St Jude RET-3 protocol (n = 23) or best clinical management (n = 37). Ototoxicity was evaluated by three different grading systems.Results: Twelve patients (20%) developed ototoxicity at some time after treatment initiation; however, ototoxicity resolved in two patients, and thus,10 patients (17%) had sustained hearing loss as documented at their most recent audiologic evaluation. Nine of these 10 patients had grade 3 or 4 ototoxicity, and nine patients were less than 6 months of age at the start of chemotherapy. Age at the start of chemotherapy was the only risk factor identified as a significant predictor of sustained hearing loss. Younger age was associated with an increased incidence of hearing loss. The different ototoxicity grading systems showed good overall agreement in the identification of patients with ototoxicity. Agreement was greatest between the Brock and Children's Cancer Group systems.Conclusion: We found that young patients with retinoblastoma who were treated with systemic carboplatin had a higher incidence of ototoxicity than previously reported. Younger patients (< 6 months of age at the start of treatment) were more likely to have ototoxicity than were older patients. Children treated with carboplatin should routinely undergo thorough, long-term audiologic monitoring.

Gangliogliomas are low-grade glioneuronal tumors of the central nervous system and the commonest cause of chronic intractable epilepsy. Most gangliogliomas (>70%) arise in the temporal lobe, and infratentorial tumors account for less than 10%. Posterior fossa gangliogliomas can have the features of a classic supratentorial tumor or a pilocytic astrocytoma with focal gangliocytic differentiation, and this observation led to the hypothesis tested in this study - gangliogliomas of the posterior fossa and spinal cord consist of two morphologic types that can be distinguished by specific genetic alterations.

Background: Patients with low‐grade gliomas (LGG), which are the most common childhood brain tumors, have excellent long‐term survival. Dissemination of LGG is rare. Robust data on the incidence, presentation, patterns of dissemination, disease behavior, outcome, and best‐management approaches do not exist. We describe 20 years of follow‐up of children with metastatic LGG.Procedure: Data collected during the period 1990–2010 were retrospectively reviewed for the following inclusion criteria: diagnosis of metastatic LGG, age younger than 21 years at initial diagnosis, and magnetic resonance imaging of the brain and/or spine at diagnosis and/or follow‐up. Patient demographics, pathology, treatment modalities, and outcome were reviewed.Results: Of 599 patients with LGG, 38 (6%) had metastatic disease at either diagnosis or follow‐up. Most tumors (87%) were located in the brain, and half of the patients had metastatic disease at presentation. The most common diagnosis was pilocytic astrocytoma (55%). Chemotherapy was the most common initial treatment modality. Median survival of the group was 6.2 years (range, 0.1–16.9 years). Fifteen (40%) patients died at a median of 6 years from diagnosis (range, 0.8–15 years). Overall survival at 5, 10, and 15 years was 80.7 ± 6.6%, 63.0 ± 10.2%, and 50.9 ± 16.0%, respectively.Conclusion: This study describes the longest follow‐up of children with metastatic LGG. LGG is underestimated and entails major morbidity and mortality. Prospective studies are needed to learn the true incidence, study the biology, and determine the best approaches to diagnosis, treatment, and follow‐up. Pediatr Blood Cancer 2015; 9999:1–9.