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Meningiomas and their WHO histological diagnostic criteria is complex, especially for grade 2 tumors presenting a interobserver discordance as high as 12.2%. The 2016 edition of the WHO Classification of CNS tumors recommended brain invasion as a stand-alone grading criterion for diagnosing an atypical grade 2 meningioma (AM). To provide an overview of the classification of 2016 WHO impact on the natural history of atypical meningioma (AM) relative to previous classification. To achieve this goal, we selected articles from the period 2017-2024 in Medline search on atypical meningiomas and analyzed them after following the following criteria: 1) reports with confirmed histopathological diagnosis according to WHO 2016 and or 2021 criteria; 2) series and case reports; 3) detailed and individualized clinical outcomes for AM; and 4) papers written in English; after that a total of 3445 patients reported in 67 manuscripts from worldwide centers from 2017 to March 2024 were analyzed. The patient's age at the time of surgery ranged from 1 month to 97 years (mean 52.28 ± 18.7 years). The most common tumor site was the convexity, accounting for 67.8%, followed by the skull base in 30.6%, ventricle in 1%, and spine in 0.6%; Gross total resection (GTR) was performed in 71.25% and subtotal resection (STR) in 28.75%; 1021 patients (29.63%) underwent adjuvant radiotherapy, and 22 patients (0.6%) were treated with adjuvant chemotherapy; tumor recurrence was reported in 1221 patients (35.44%) and 859 deaths (24.93%). 1) AM prevalence in females; 2) AM age distribution similar to the distribution of meningiomas in general; 3) AM recurrence rate of 35.44%, despite the high rate of GTR, which was higher than previously reported; 4) deepening knowledge in molecular mechanism of tumor progression will provide alternative therapeutic approaches for AM., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

In metazoans, microRNAs (miRNAs) are essential regulators of gene expression, affecting critical cellular processes from differentiation and proliferation, to homeostasis. During miRNA biogenesis, the miRNA strand that loads onto the RNA-induced Silencing Complex (RISC) can vary, leading to changes in gene targeting and modulation of biological pathways. To investigate the impact of these "arm switching" events on gene regulation, we analyzed a diverse range of tissues and developmental stages in zebrafish by comparing 5p and 3p arms accumulation dynamics between embryonic developmental stages, adult tissues, and sexes. We also compared variable arm usage patterns observed in zebrafish to other vertebrates including arm switching data from fish, birds, and mammals. Our comprehensive analysis revealed that variable arm usage events predominantly take place during embryonic development. It is also noteworthy that isomiR occurrence correlates to changes in arm selection evidencing an important role of microRNA distinct isoforms in reinforcing and modifying gene regulation by promoting dynamics switches on miRNA 5p and 3p arms accumulation. Our results shed new light on the emergence and coordination of gene expression regulation and pave the way for future investigations in this field., Competing Interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article., (© The Author(s) 2024.)

To describe the natural history of spinal gangliogliomas (GG) in order to determine the most appropriate neuro-oncological management. A Medline search for relevant publications up to July 2023 using the key phrase "ganglioglioma spinal" and "ganglioglioma posterior fossa" led to the retrieval of 178 studies. This corpus provided the basis for the present review. As an initial selection step, the following inclusion criteria were adopted: (i) series and case reports on spinal GG; (ii) clinical outcomes were reported specifically for GG; (iii) GG was the only pathological diagnosis for the evaluation of the tumor; (iv) papers written only in English was evaluated; and (v) papers describing each case in the series were included. The World Health Organization (WHO) 2021 grading criteria for gangliogliomas were applied. A total of 107 tumors were evaluated (63 from male patients and 44 from female patients; 1.43 male/1.0 female ratio, mean age 18.34 ± 15.84 years). The most common site was the cervical spine, accounting for 43 cases (40.18%); GTR was performed in 35 cases (32.71%) and STR in 71 cases (66.35%), while this information was not reported in 1 case (0.94%). 8 deaths were reported (7.47%) involving 2 males (25%) and 6 females (75%) aged 4-78 years (mean 34.27 ± 18.22) years. GGs located on the spine displayed the same gender ratio as these tumors in general. The most frequent symptom was pain and motor impairment, while the most prevalent location was the cervical spinal cord. GTR of the tumor posed a challenge for neurosurgeons, due to the difficulty of resecting the lesion without damaging the spinal eloquent area, explaining the lower rate of cure for this tumor type., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Objective: The authors searched for genetic and transcriptional signatures associated with tumor progression and recurrence in their cohort of patients with meningiomas, combining the analysis of targeted exome, NF2-LOH, transcriptome, and protein expressions., Methods: The authors included 91 patients who underwent resection of intracranial meningioma at their institution between June 2000 and November 2007. The search of somatic mutations was performed by Next Generation Sequencing through a customized panel and multiplex ligation-dependent probe amplification for NF2 loss of heterozygosity. The transcriptomic profile was analyzed by QuantSeq 3' mRNA-Seq. The differentially expressed genes of interest were validated at the protein level analysis by immunohistochemistry., Results: The transcriptomic analysis identified an upregulated set of genes related to metabolism and cell cycle and downregulated genes related to immune response and extracellular matrix remodeling in grade 2 (atypical) meningiomas, with a significant difference in recurrent compared with nonrecurrent cases. EZH2 nuclear positivity associated with grade 2, particularly with recurrent tumors and EZH2 gene expression level, correlated positively with the expression of genes related to cell cycle and negatively to genes related to immune response and regulation of cell motility., Conclusions: The authors identified modules of dysregulated genes in grade 2 meningiomas related to the activation of oxidative metabolism, cell division, cell motility due to extracellular remodeling, and immune evasion that were predictive of survival and exhibited significant correlations with EZH2 expression.

Background: The correlation between angioarchitecture and clinical presentation of brain arteriovenous malformation (bAVM) remains a subject of debate., Objective: The main purpose of the present study was to assess the correlation between angioarchitectural characteristics of bAVM and clinical presentation., Methods: A retrospective review of all consecutive patients presenting a bAVM who underwent a cerebral angiography at Beneficencia Portuguesa Hospital in São Paulo between January 2006 and October 2016 was carried out. Patients were divided in five groups: group 1 - hemorrhage; group 2 - seizure; group 3 - headache; group 4 - progressive neurological deficits (PND); group 5 - incidental)., Results: A total of 183 patients were included, with group 1 comprising 56 cases, group 2 49 cases, group 3 41 cases, group 4 28 cases, and group 5 9 cases. Regarding hemorrhage presentation, a statistical correlation was observed with female gender (P < 0.02), Spetzler-Martin 3B (P < .0015), and lesions with low flow (P < 0.04). A positive association was found between group 2 and age less than 36 years (P < 0.001), male sex (P < 0.018), presence of superficial lesions not classified as SM 3B (P < 0.002), presence of venous ectasia (p <0.03), and arterial steal phenomenon (P < 0.03). Group 4 was associated with older age (P < 0.01)., Conclusions: Angioarchitectural characteristics can be correlated with some clinical presentations as well as with some clinical data, making it possible to create predictive models to differentiate clinical presentations.

ATRX-DAXX-H3.3 chromatin remodeler complex is a well known epigenetic factor responsible for the heterochromatin maintenance and control. ATRX is an important nucleosome controller, especially in tandem repeat regions, and DAXX is a multi-function protein with particular role in histone H3.3 deposition due to its chaperone characteristic. Abnormalities in this complex have been associated with telomere dysfunction and consequently with activation of alternative lengthening of telomeres mechanism, genomic instability, and tumor progression in different types of cancer. However, the characterization of this complex is still incomplete in meningioma. We analyzed ATRX, DAXX and H3.3 expressions and the telomere length in a cohort of meningioma of different malignant grades. We observed ATRX upregulation at gene and protein levels in grade II/III meningiomas. A low variability of telomere length was observed in meningiomas across different ages and malignant grades, in contrast to the shortening of telomere length with aging in normal controls., (Copyright © 2021 Elsevier B.V. All rights reserved.)

Objective: To review the data published on the subject to create a more comprehensive natural history of the haemorrhagic onset of meningiomas (IVMs)., Patients and Methods: A Medline search up to June 2020, using the search term "bleeding meningioma," returned 136 papers. As a first selection step, we adopted the following inclusion criteria: series and case reports about bleeding meningioma. Papers written in other languages but with abstracts written in English were also evaluated., Results: A total of 190 tumours were evaluated, specifically 109 tumours from female patients and 81 tumours from male patients with a ratio of 1.34 female to 1.0 male (mean age of 54.86 ± 16.1years old). The majority were located in the convexity (129-67.9 %). Among the 190 tumours evaluated, 171 patients (90 %) presented with GI tumours, with a predominance of the meningothelial subtype (32.6 %). Nine patients (4.7 %) presented with grade GII tumours, and 10 (5.3 %) presented with GIII tumours. The most prevalent type was intracerebral haemorrhage (ICH) at 50 %, followed by subdural at 27.36 %; the mortality rate was 13.1 % (25 deaths), the distribution of both location (prevalence of convexity: 18-72 %) and histopathology (grade 1: 22-88 %)., Conclusion: These tumours follow the histopathological distribution of meningiomas, in general. The age distribution shows prevalence among the adult population but with a greater proportion in the elderly. The fact that the overwhelming majority of cases involve meningiomas with a benign histological subtype is noteworthy. Another relevant factor observed is that most reports are from Asian origin., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Review the data published on the subject to create a more comprehensive natural history of intraventricular meningiomas (IVMs). A Medline search up to March 2018 using "intraventricular meningioma" returned 98 papers. As a first selection step, we adopted the following inclusion criteria: series and case reports about IVMs, as well as papers written in other languages, but abstracts written in English were evaluated. Six hundred eighty-one tumors were evaluated from 98 papers. The majority of the tumors were located in the lateral ventricles (602-88.4%), fourth ventricle (59-8.7%), and third ventricle (20-2.9%). These tumors accounted for a mortality rate of 4.0% (25 deaths) and a recurrence rate of 5.3% (26 recurrences). The majority of the tumors were grade I (89.8%) and consisted of the following subtypes: fibrous, 39.7% (n = 171); transitional, 22.0% (n = 95); meningothelial, 18.6% (n = 80); angiomatosus, 3.2% (n = 14); psammomatous, 2.6% (n = 11); and others, 13.9% (n = 60). Forty-five patients (7.4%) presented with grade II (GII) tumors, and 17 patients (2.8%) presented with grade III (GIII) tumors. These tumors follow the histopathological distribution of meningiomas in general, with the exception of the higher prevalence of the fibrous subtype, possibly due to its embryonic origin. Recurrence and mortality were lower than in other localizations likely due to a complete surgical resection rate than in the convexity and skull base, which suggests that GTR is the gold standard for the management of IVMs.

Objective: The aim of the present study was to analyze if the pathway Skp2-p27-cyclin E1 could also be a tumor progression marker for meningiomas., Patients and Methods: We used quantitative real-time PCR to assess the relative expression levels of the genes coding for cyclin E1 (CCNE1), Skp2 (SKP2), and p27 (P27). The expression levels were compared in grades I to III meningiomas and among different histological subtypes of grade I meningiomas., Results: Anaplastic meningiomas accounted for 4.9%, atypical meningiomas for 23.5% and grade I meningiomas for 71.6%.CCNE1 expression level was significantly higher in grade II compared to grade I meningiomas (p = 0.0027), and its expression level reliably predicts grade II meningiomas (ROC AUC = 0.731, p = 0.003). CCNE1 expression also correlated with SKP2 and P27 expression levels in grade I meningiomas (r = 0.539, p < 0.0001 and r = 0.687, p = <0.0001, respectively for CCNE1/SKP2 and CCNE1/P27, Spearman's test). Fibrous subtype among grade I meningiomas presented the highest expression levels of CCNE1, SKP2 and P27. Higher expression of cyclin E1 protein was detected in the nuclei of atypical meningiomas compared to grade I meningiomas., Conclusions: CCNE1 expression level predicts meningioma malignancy, and the fibrous subtype presents the highest gene expression levels among grade I meningiomas., Competing Interests: Declaration of Competing Interest The authors have no personal financial or institutional interest in any of the drugs, materials, or devices described in this article., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Evaluate whether radiotherapy (RT) after the neurosurgical treatment of atypical meningiomas (AM) has an impact on the reduction rate of recurrence. A Medline search through October 2017 using "atypical meningioma" returned 1277 papers for initial review. Inclusion criteria were as follows. We analyzed the database and included articles in which the anatomic pathological classification of atypical meningiomas was in accordance with WHO 2007 or WHO 2016 criteria, patients > 18 years of age, and there was postoperative external beam radiation to the tumor bed. Exclusion criteria were WHO grade I or III meningioma, patients who underwent whole-brain radiation, RT used as salvage therapy for recurrence, palliative dose of RT (< 45 Gy), recurrent AMs, and multiple AMs. Papers reporting outcomes in which atypical and anaplastic meningiomas were analyzed together were rejected, as were papers with small samples that may compromise evaluation. After filtering our initial selection, only 17 papers were selected. After reviewing the seventeen articles including a total of 1761 patients (972 female and 799 male; 1.21 female/1.0 male), the difference in proportion of tumor recurrence between patients with and without radiotherapy after neurosurgical procedure was 1.0448, 95% CI [0.8318 to 1.3125], p value = 0.7062. On the basis of this review, there is no evidence to suggest that RT decreases the rate of recurrence in patients with atypical meningiomas.

Meningiomas, tumors that originate from meningothelial cells, account for approximately 30% of all new diagnoses of central nervous system neoplasms. According to the 2016 WHO classification of central nervous system tumors meningiomas are classified into three grades: I, II, and III. Past studies have shown that the risk of meningiomas recurrence is strongly correlated with the molecular profile of the tumor. Extensive whole-exome or whole-genome sequencing has provided a large body of information about the mutational landscape of meningiomas. However, such a stratification of meningiomas based on mutational analysis alone has been proven not to satisfy the clinical need for distinction between patients who need (or do not need) an adjuvant treatment. Combined analysis of exome, transcriptome, methylome and future approaches for epigenetic aspects in meningiomas may allow researchers to unveil a more comprehensive understanding of tumor progression mechanisms and, consequently, a more personalized clinical approach for patients with meningioma. A better understanding of the genetics and clinical behavior of high-grade meningiomas is mandatory in order to better design future clinical trials. By studying the mechanisms underlying these new tumorigenesis pathways, we should be able to offer personalized chemotherapy to patients with surgery and radiation-refractory meningiomas in the near future. The purpose of this article is to accurately bring the compilation of this information, for a greater understanding of the subject., (Copyright © 2018 Elsevier B.V. All rights reserved.)

Objective: Isolated dissecting spinal aneurysms are rare lesions that pose a therapeutic challenge. We report our experience in the management of four patients with dissecting spinal aneurysms., Methods: After institutional review board approval was obtained, the neurointerventional databases of the two participating institutions were retrospectively reviewed. Aneurysms in the anterior spinal artery, posterior spinal artery, or in a radiculomedullary artery were included in the analysis. Flow-related aneurysms were excluded. Data on clinical presentation, hemorrhage location, aneurysm size, location, angiographic follow-up, re-hemorrhage, and clinical outcome were obtained and analyzed., Results: Four patients with five spinal dissecting aneurysms met the inclusion criteria. There were two women and the mean age was 63 years (range 36-64). All patients presented with hemorrhage. Three radiculomedullary arteries in two different patients had one lesion each; the other two involved the anterior spinal artery and a posterior spinal artery in different patients. All four patients were managed conservatively. Follow-up angiography (5.5 months) demonstrated occlusion of all five aneurysms. There was no re-hemorrhage and all patients had a good outcome at the last follow-up (modified Rankin Scale score 0-2)., Conclusions: Conservative management of ruptured spontaneous dissecting spinal aneurysms is a reasonable therapeutic option capable of achieving favorable angiographic and clinical outcomes., Competing Interests: Competing interests: None declared., (© Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

Objective: The aim of this study was to describe 27 patients with 32 web vessels (WVs) from our practice and provide a review of the literature regarding imaging diagnostics, clinical presentation, and treatment of WVs., Methods: A MEDLINE search was performed using combinations of the following terms: "stroke in young adults," "web," "recurrent stroke," "diaphragm-like," "atypical fibromuscular hyperplasia," "atypical fibromuscular dysplasia," "septal fibromuscular dysplasia," "septa," "diaphragms," "pseudovalvular folds," and "carotid diaphragm." Our series was added in this review., Results: According to location, there were 88 carotid WVs (91.66%), 7 vertebral WVs (7.3%), and 1 subclavian WV (1.04%). According to clinical presentation, the reported WVs caused stroke in 81 cases (84.38%). Treatment was supportive care in 46 cases (47.91%), surgery in 33 cases (35.41%), and an endovascular procedure through angioplasty and stenting in 17 cases (17.7%). Variables such as age (P = 0.7565), sex (P = 0.6912), and location (P = 0.7993) were not shown to be risk factors for stroke in these patients., Conclusions: Although few cases have been reported in the literature, the stroke rate in patients with WVs is high. Endovascular treatment is presented as an effective form of treatment with no associated morbidities or recurrences of ischemic events., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Objective: To analyze the surgical and oncologic treatment of a Brazilian cohort of patients with grade II and III meningioma at a follow-up time of 15 years to get an overview of these patients' outcomes., Methods: Cross-sectional study of 43 patients (26 women, 17 men; age range 20 to 83 years; average 57.72 ± 14.54) operated on from 2000 to 2014 at a single institution, with the neuropathologic diagnosis of meningioma grade II (39 patients) and grade III (4 patients)., Results: Radiotherapy: 24 patients (55.81%) underwent radiotherapy; the time between the surgical procedure and the beginning of radiotherapy was 5 months; 7 patients with a diagnosis of AM underwent a new surgical procedure, albeit of adjuvant therapy, because of tumor recurrence, and only 3 of them underwent radiotherapy after the first resection. Mortality: in total, 19 deaths (44.18%) were identified in this sample: 15 (38.46%) with GII and 4 (100%) with GIII. The 10-year survival was expected in 35% of GII patients and 0% of GIII patients., Conclusion: Surgery is still the main form of treatment and the mainstay for prolonging survival. Radiotherapy is still controversial; however, we observed its positive impact on recurrence and progression-free survival., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Background and Purpose: This study is a homogeneous series of circumferential unruptured intracranial aneurysms with large necks treated with stent-assisted coil embolization. Our purpose was to demonstrate which value of packing density is required to produce a durable occlusion., Materials and Methods: We retrospectively evaluated all patients with unruptured intracranial aneurysms who were treated with stent-assisted coil embolization having late angiographic control between 2004 and 2014, in a single large cerebrovascular referral center. To calculate the packing density, aneurysm volume, and coil volume, we used an on-line system., Results: In 49 circumferential unruptured intracranial aneurysms treated with stent-assisted coil embolization, 38.7% ( n = 19) had complete occlusion in the immediate control. Of those with incomplete occlusion, 80% ( n = 24) progressed to complete occlusion in the late angiographic follow-up. At late angiographic control, 87.7% ( n = 43) of aneurysms were completely occluded. All aneurysms with a packing density of ≥19% were completely occluded. Packing density was the only statistically significant predictor of complete occlusion. None of the aneurysms with complete occlusion at immediate control or at late angiographic control had recurrence., Conclusions: In circumferential aneurysms treated with stent-assisted coil embolization, packing density is the main predictor of complete occlusion. In this type of aneurysm, a packing density of ≥19% was enough to reach complete occlusion; knowing this is important to avoid higher packing densities that have more risk., (© 2017 by American Journal of Neuroradiology.)

Objective: The medical literature still lacks information about the impact of surgery and adjuvant treatment on the life of patients with meningioma. The clinical outcome, timing of tumor recurrence, and causes of death are often overlooked. This study evaluates these data taking into account tumor localization and histologic grade., Methods: The article is a cross-sectional study of patients operated on between 2000 and 2014 in a single institution. The series has 593 adult patients (442 females and 151 males) and follow-up of 68.8 ± 48.9 months. Imaging of 434 patients was reviewed and 379 patients/families interviewed., Results: Sixty-eight deaths were related to tumor treatment/progression and 36 to other causes. After 2 years of surgery, deaths not related to tumor were 7 times more frequent than were tumor-related deaths (odds ratio, 7.1; 95% confidence interval, 2.8-19.5; P < 0.0001). Ten-year survival was expected in 85% of patients with grade I (GI) meningioma, 35% of patients with atypic (GII) meningioma, and 0% of patients with anaplastic (GIII) meningioma. Convexity tumors had about half the risk of recurrence compared with other localizations (odds ratio, 0.4; 95% confidence interval, 0.27-0.67; P = 0.0002). In GI meningioma, recurrence was neither related to death nor to impairment of independent life. All patients with GII and GIII meningioma who had recurrence died. 96.3% of interviewees reported neurologic improvement or stability after the surgery., Conclusions: Histologic grade is the most important factor for long-term survival. Complete resection has to be pursued in GII and GIII meningioma but must be carefully weighed against morbidities in GI meningioma., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Background: Treatment of ophthalmic segment aneurysms (OSA) remains challenging. Flow diverter stents (FDS) have evolved as a promising endovascular treatment option for aneurysms of the internal carotid artery and are associated with high occlusion rates and a favorable morbidity and mortality profile., Objective: To determine safety and efficacy of FDS for OSA in a large, multicenter cohort., Methods: A retrospective analysis of prospectively maintained databases of 127 consecutive patients harboring 160 OSA treated with FDS was performed. Aneurysms were classified based on location and morphology. Follow-up with digital subtraction angiography (DSA) was performed 6 to 18 months after treatment., Results: Follow-up DSA was available for 101 (63.1%) aneurysms with a mean follow-up of 18 months. Complete occlusion was observed in 90 aneurysms (89.1%), near-complete occlusion (>95%) in 3 (3%), and incomplete occlusion (<95%) in 8 aneurysms (7.9%). One aneurysm was retreated with another FDS (0.9%). No risk factors for incomplete occlusion were identified. The OA was occluded at the latest follow-up in 6 cases (7.1%). Permanent morbidity occurred in 4 patients (3.1%), and there was no mortality related to the FDS procedure., Conclusion: Treatment of OSA with FDS was found to be safe and effective. The retreatment rate was extremely low and aneurysms that occluded did not reanalyze., (Copyright © 2016 by the Congress of Neurological Surgeons.)

Objective: The objective of this study is to present a case series of nonmissile penetrating (NMP) injuries and to establish a workflow for an uncommon mechanism of traumatic head injury through the analysis of each case, classification of the type of lesion, management, and outcome score at follow-up., Methods: From January 1991 to December 2008, 36,000 patients presenting with traumatic brain injury (TBI) were admitted in the Department of Neurosurgery, Hospital Antônio Targino, Campina Grande-PB, Brazil. From these patients, 11 presenting with lesions caused by NMP objects were selected., Results: Among the 11 patients, 9 were men and 2 were women. Their ages ranged from 7 to 74 years old (mean age ± SD, 29.1 ± 22.99 years). All patients underwent neuroradiologic evaluation. The entry point was classified as natural (orbit) or artificial (skull transfixation), and we also divided the patients presenting with secondary parenchymal or vascular damage from those presenting with only lesions caused by the primary penetration into the cranium and meninges. All patients were neurosurgically treated with removal of the foreign body through craniotomy, except the patient whose object (pen) was removed without craniotomy with local anesthesia. Glasgow Coma Scale (GCS) score on admission was a statistically significant factor on prognosis, and any patient who presented with a GCS score of 15 evolved satisfactorily, and there were no deaths in this group of patients (P = 0.04)., Conclusions: TBIs caused by NMP objects are unusual and caused by aggression, self-inflicted harm (in the case of psychiatric patients), and accident. The foreign body may enter into the skull through a natural hole (orbit, nose, mouth, or ear) or crosses the skull, causing a fracture and creating an artificial hole. Preoperative neuroradiologic assessment is paramount for the correct neurosurgical approach. The main prognostic factor for these patients is the GCS score at admission., (Copyright © 2016 Elsevier Inc. All rights reserved.)