Multinodular and vacuolating neuronal tumor (MVNT) is a new pattern of neuronal tumour included in the recently revised WHO 2016 classification of tumors of the CNS. There are 15 reports in the literature to date. They are typically associated with late onset epilepsy and a neoplastic vs. malformative biology has been questioned. We present a series of ten cases and compare their pathological and genetic features to better characterized epilepsy‐associated malformations including focal cortical dysplasia type II (FCDII) and low‐grade epilepsy‐associated tumors (LEAT). Clinical and neuroradiology data were reviewed and a broad immunohistochemistry panel was applied to explore neuronal and glial differentiation, interneuronal populations, mTOR pathway activation and neurodegenerative changes. Next generation sequencing was performed for targeted multi‐gene analysis to identify mutations common to epilepsy lesions including FCDII and LEAT. All of the surgical cases in this series presented with seizures, and were located in the temporal lobe. There was a lack of any progressive changes on serial pre‐operative MRI and a mean age at surgery of 45 years. The vacuolated cells of the lesion expressed mature neuronal markers (neurofilament/SMI32, MAP2, synaptophysin). Prominent labelling of the lesional cells for developmentally regulated proteins (OTX1, TBR1, SOX2, MAP1b, CD34, GFAPδ) and oligodendroglial lineage markers (OLIG2, SMI94) was observed. No mutations were detected in the mTOR pathway genes, BRAF, FGFR1 or MYB. Clinical, pathological and genetic data could indicate that MVNT aligns more with a malformative lesion than a true neoplasm with origin from a progenitor neuro‐glial cell type showing aberrant maturation.
Unlocking the secrets of the brain is a task fraught with complexity and challenge – not least due to the intricacy of the circuits involved. With advancements in the scale and precision of scientific technologies, we are increasingly equipped to explore how these components interact to produce a vast range of outputs that constitute function and disease. Here, an insight is offered into key areas in which the marriage of neuroscience and nanotechnology has revolutionized the industry. The evolution of ever more sophisticated nanomaterials culminates in network-operant functionalized agents. In turn, these materials contribute to novel diagnostic and therapeutic strategies, including drug delivery, neuroprotection, neural regeneration, neuroimaging and neurosurgery. Further, the entrance of nanotechnology into future research arenas including optogenetics, molecular/ion sensing and monitoring, and piezoelectric effects is discussed. Finally, considerations in nanoneurotoxicity, the main barrier to clinical translation, are reviewed, and direction for future perspectives is provided.
This retrospective series highlights the poor prognosis associated with the diagnosis of malignant meningioma. Complete or subtotal resection may prolong the patients' survival. We could not confirm the usefulness of postoperative radiotherapy.
BackgroundElective intraparenchymal intracranial pressure (ICP) monitoring is useful for the diagnosis and treatment of hydrocephalus and cerebrospinal fluid (CSF) disorders. This retrospective study analyzes median ICP and pulse amplitude (PA) recordings in neurosurgically naïve patients undergoing elective ICP monitoring for suspected CSF disorders.MethodsRetrospective review of prospectively collated database of neurosurgically naïve patients undergoing elective ICP monitoring for suspected hydrocephalus and CSF disorders. Following extraction of the median ICP and PA values (separated into all, day and night time recordings), principal component analysis (PCA) was performed to identify the principal factors determining the spread of the data. Exploratory comparisons and correlations of ICP and PA values were explored, including by post hoc diagnostic groupings and age.ResultsA total of 198 patients were identified in six distinct diagnostic groups (n = 21–47 in each).The PCA suggested that there were two main factors accounting for the spread in the data, with 61.4% of the variance determined largely by the PA and 33.0% by the ICP recordings.Exploratory comparisons of PA and ICP between the diagnostic groups showed significant differences between the groups. Specifically, significant differences were observed in PA between a group managed conservatively and the Chiari/syrinx, IIH, and NPH/LOVA groups and in the ICP between the conservatively managed group and high-pressure, IIH, and low-pressure groups. Correlations between ICP and PA revealed some interesting trends in the different diagnostic groups and correlations between ICP, PA, and age revealed a decreasing ICP and increasing PA with age.ConclusionsThis study provides insights into hydrodynamic disturbances in different diagnostic groups of patients with CSF hydrodynamic disorders. It highlights the utility of analyzing both median PA and ICP recordings, stratified into day and night time recordings.
BACKGROUND In contrast to benign meningiomas, malignant meningiomas (MM) are rare and associated with an unfavourable prognosis. Reports on MM concern fairly small cohorts, often comprising less than 30 cases. OBJECTIVE To describe the outcome MM and identify factors that may influence survival. METHODS Pathology reports and clinical data of 178 patients treated between 1989 and 2017 for a MM at 6 different international institutions were retrospectively reviewed. Seventy-six patients (42.7%) had a previous history of grade I or grade II meningioma. The patients underwent a total of 380 surgical resections and 72.5% received radiotherapy. Median follow-up was 4.5 yr. RESULTS At data collection, 111 patients were deceased (63.4%) and only 23 patients (13.7%) were alive without any residual tumor on the most recent scan. Median overall survival was 2.9 yr, 95% confidence interval [CI; 2.4, 4.5]. Overall survival rates at 1, 5, and 10 yr, respectively, were: 77.7%, 95% CI [71.6, 84.3], 40%, 95% CI [32.7, 49], and 27.9%, 95% CI [20.9, 37.3]. In the multivariable analysis, age at MM surgery <65 yr (hazard ratio [HR] = 0.44, 95% CI [0.29, 0.67], P < .001), previous benign or atypical meningioma surgery (HR = 1.9, 95% CI [1.23, 2.92], P = .004), completeness of resection (HR = 0.51, 95% CI [0.34, 0.78], P = .002), and adjuvant radiotherapy (HR = 0.64, 95% CI [0.42, 0.98], P = .039) were established as independent prognostic factors for survival. CONCLUSION This large series confirms the poor prognosis associated with MM, the treatment of which remains challenging. Patients under 65-yr-old with primary MM may live longer after complete resection and postoperative radiotherapy. Even with aggressive treatments, local control remains difficult to achieve.
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