Journal of neuro-oncology
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Journal of neuro-oncology · Jan 2014
Benign whole body tumor volume is a risk factor for malignant peripheral nerve sheath tumors in neurofibromatosis type 1.
The purpose of this study is to determine whether benign whole body tumor volume of plexiform neurofibromas (PNs) is a risk factor for malignant peripheral nerve sheath tumors (MPNST) in individuals with neurofibromatosis type 1 (NF1). Thirty-one NF1 patients with MPNSTs and 62 age- and sex-matched NF1 patients without MPNSTs, who had undergone whole body magnetic resonance imaging (MRI) were analyzed for benign whole body tumor volume. Mann-Whitney U test, Wilcoxon signed ranks test, Fisher's exact test (two-tailed), and logistic regression analysis were used for statistical analysis. ⋯ Higher numbers of PNs, larger whole body benign tumor volume, and younger age are important risk factors for MPNST. We identified a subgroup of patients with MPNST without internal PN on MRI and the lack of correlation of MPNST development with tumor burden in older patients. These findings may alter our belief that all MPNSTs arise from pre-existing PNs and suggest that surveillance MRI based on clinical suspicion may be warranted in older patients, respectively.
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Journal of neuro-oncology · Jan 2014
Long-term evaluation of cognition after glioma surgery in eloquent areas.
Preservation of cognition is an important outcome measure in eloquent area glioma surgery. Glioma patients may have pre-operative deficits in one or more cognitive domains which could deteriorate post-operatively. It is assumed that these impairments recover within 3 months; some studies however, still detected cognitive decline. ⋯ The findings underline the importance of cognitive testing at longer term post-operatively, as cognitive recovery took longer than 3 months, especially within the language domain. However, this longitudinal follow-up study showed that glioma surgery is possible without major long-term damage of cognitive functions. Tumor characteristics and EOR are no additional risk factors for cognitive outcome.
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Journal of neuro-oncology · Dec 2013
Comparative StudyNatural history and role of radiation in patients with supratentorial and infratentorial WHO grade II ependymomas: results from a population-based study.
Patients with World Health Organization (WHO) grade II supratentorial ependymomas are commonly observed after gross total resection (GTR), although supporting data are limited. We sought to characterize the natural history of such tumors. We used the Surveillance, Epidemiology, and End Results program to identify 112 patients ages 0-77 diagnosed with WHO grade II ependymomas between 1988 and 2007, of whom 63 (56 %) and 49 (44 %) had supratentorial and infratentorial primaries, respectively. ⋯ Among the whole cohort, on both univariable and multivariable regression, extent of resection was predictive of ESM, while tumor location and use of radiation were not. After GTR, patients with WHO grade II supratentorial ependymomas have a very favorable natural history with low associated cancer-specific mortality. Observation, with radiation reserved as a salvage option, may be a reasonable postoperative strategy in this population.
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Journal of neuro-oncology · Dec 2013
Supratentorial gross-totally resected non-anaplastic ependymoma: population based patterns of care and outcomes analysis.
Observation following gross-total resection (GTR) for non-anaplastic supratentorial ependymomas is often advocated based on small, retrospective series. The purpose of this study is to perform a population-based analysis to examine outcomes for this rare cohort of low-risk patients. A retrospective analysis was conducted utilizing the Surveillance, Epidemiology and End Results Program of the United States National Cancer Institute. ⋯ Age ≥18 years correlated with worse OS (HR 4.01 [1.45-11.11]; p = 0.008) and CSS (HR 2.86 [0.99-8.31]; p = 0.05). RT did not impact outcome for this low-risk cohort of patients. Older age correlates with poor prognosis.
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Journal of neuro-oncology · Dec 2013
The differential diagnosis of pilocytic astrocytoma with atypical features and malignant glioma: an analysis of 16 cases with emphasis on distinguishing molecular features.
Rare pilocytic astrocytomas (PA) have atypical histologic and clinicoradiologic features that raise the differential diagnosis of glioblastoma. Whether ancillary studies can supplement histopathologic examination in placing these cases accurately on the spectrum of WHO Grade I PA to higher-grade glioma is not always clear, partly because these cases are not common. Here, ten PAs with atypical clinicoradiologic and histologic features and six pediatric glioblastoma multiforme (pGBMs) were analyzed for BRAF V600E, IDH1, IDH2, and TP53 mutations. ⋯ IDH1 and IDH2 were wild type in all cases, consistent with earlier findings that IDH mutations are not typical in high-grade gliomas of patients ≤14 years of age. Immunohistochemical studies showed substantial overlap in Ki-67 labeling indices, an imperfect correlation between p53 labeling and TP53 mutation status, and complete p16 loss in only two pGBMs but in no PAs. These results suggest that (a) BRAF-KIAA1549 fusion may be common in PAs with atypical clinicoradiologic and histologic features, including those at extracerebellar sites, (b) BRAF V600E mutation is uncommon in extracerebellar PAs, and (c) TP53 mutation analysis remains a valuable tool in identifying childhood gliomas that will likely behave in a malignant fashion.