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Background: Spheno-orbital meningioma (SOM) represents a unique variant of sphenoid wing meningiomas, distinguished by its propensity for bone infiltration and cranio-orbital involvement. SOM exhibits a considerable incidence of misdiagnosis and recurrence., Purposes: To elucidate the clinical, radiological, and pathological characteristics of SOM., Methods: Review of electronic medical records, histopathology, radiological images and follow-up information of 100 SOM patients., Results: Of the 100 patients (28 males, 72 females) with SOM, mean age was 46.8 ± 12.6 years and prevalent symptoms were proptosis (99%). All the CT scans showed hyperostosis with 89.3% of the hyperostosis having an irregular edge. In MRI scans, dural tail sign was observed across all patients and the cranio-orbital tumors often penetrated temporal muscle (74.1%), extraocular muscle (74.1%) and lacrimal gland (63%). All the 100 patients underwent surgical intervention, and among them, 62 individuals received postoperative radiotherapy. Grade I resections had a lower recurrence rate(16.7%), which further decreased with the addition of radiotherapy(13.9%). In contrast, all patients with grade II or higher grade resections without radiotherapy experienced recurrence, indicating a higher risk associated with less complete tumor removal. The pathological examination revealed that intraorbital sections exhibited comparable tumor density to intraorbital SOM tumors, along with increased fibrous density but decreased vascular distribution., Conclusions: Radiological characteristics of SOM included cranio-orbital tumors, hyperostosis of the sphenoid wing with an irregular edge, and dural tail sign. Combination of gross total resection and adjuvant radiotherapy was recommended to minimize recurrence rate. Intracranial SOM tumors tended to be softer and more bleed-prone than intraorbital sections, necessitating surgical precision., (© 2024. The Author(s).)

Purpose: This study aimed to examine the effect of postoperative radiotherapy on survival outcomes in patients with malignant meningiomas., Methods: We identified patients with malignant meningioma diagnosed between 2007 and 2018 using the Taiwan Cancer Registry and followed them up using the death registry. Survival was compared between patients with and without adjuvant radiotherapy. The potential confounding factors evaluated in this study included age, sex, comorbidities, and the Charlson Comorbidity Index (CCI)., Results: The analysis included 204 patients; 94 (46%) received adjuvant radiotherapy. The two groups had similar sex distributions (p = 0.53), mean age (p = 0.33), histologic subtype (p = 0.13), and CCI (p = 0.62). The prognosis of malignant meningioma was poor, with a median overall survival (OS) of 2.4 years. The median OS was 3.0 years (interquartile range (IQR) [1.4-6.1], and 2.0 years (IQR [0.5-3.9]) in the radiotherapy and non-radiotherapy groups, respectively (p = 0.001). However, Kaplan-Meier curves with the log-rank test showed no significant difference in OS between the two groups (p = 0.999). Controlling for age group, sex, histologic subtype, treatment, comorbidities, and CCI, adjuvant radiotherapy did not impart a survival benefit (hazard ratio [HR] = 0.87; 95% confidence interval [CI]: 0.6‒1.26); however, only factor of higher comorbidity score (HR = 2.03, 95%CI: 1.04‒3.94) was associated with unfavorable survival., Conclusion: This population-based retrospective analysis suggests that the role of radiotherapy remains unclear and underscores the need for randomized clinical trials to assess the usefulness of adjuvant radiotherapy in malignant meningioma., (© 2024. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Purpose: To report the outcomes of a large series of intracranial meningiomas (IMs) submitted to proton therapy (PT) with curative intent., Methods: We conducted a retrospective analysis on all consecutive IM patients treated between 2014 and 2021. The median PT prescription dose was 55.8 Gy relative biological effectiveness (RBE) and 66 GyRBE for benign/radiologically diagnosed and atypical/anaplastic IMs, respectively. Local recurrence-free survival (LRFS), distant recurrence-free survival (DRFS), overall survival (OS), and radionecrosis-free survival (RNFS) were evaluated with the Kaplan-Meier method. Univariable analysis was performed to identify potential prognostic factors for clinical outcomes. Toxicity was reported according to the latest Common Terminology Criteria for Adverse Events (CTCAE) version 5.0., Results: Overall, 167 patients were included. With a median follow-up of 41 months (range, 6-99), twelve patients (7%) developed tumor local recurrence after a median time of 39 months. The 5-year LRFS was 88% for the entire cohort, with a significant difference between benign/radiologically diagnosed and atypical/anaplastic IMs (98% vs. 47%, p < 0.001); this significant difference was maintained also for the 5-year OS and the 5-year DRFS rates. Patients aged ≤ 56 years reported significantly better outcomes, whereas lower prescription doses and skull base location were associated with better RNFS rates. Two patients experienced G3 acute toxicities (1.2%), and three patients G3 late toxicities (1.8%). There were no G4-G5 adverse events., Conclusion: PT proved to be effective with an acceptable toxicity profile. To the best of our knowledge this is one of the largest series including IM patients submitted to PT., (© 2024. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Despite common histogenesis meningiomas have a wide morphologic spectrum, and the World Health Organization (WHO) recognizes 15 subtypes. They are the most common brain tumour in adults and typically have an extra-axial location. Although there have been important advances in the molecular biology of meningiomas its diagnosis is based on histopathologic features. The great majority are benign WHO grade 1 tumours. There are specific criteria for assigning WHO grade 2 and 3 that can be applied to all meningioma subtypes. Regardless of these criteria, chordoid and clear cell morphologic subtypes are considered grade 2. WHO grade 3 tumours exhibit a very high mitotic index, frank anaplasia or specific molecular abnormalities. The impressive morphologic diversity shown by meningiomas makes them a diagnostic challenge, which can be even greater in intraoperative studies. The focus of this article is to describe and illustrate their main cytologic features, with emphasis on the most infrequent subtypes., (© 2023 John Wiley & Sons Ltd.)

Aims: WHO Grade 3 (G3) meningiomas are rare tumours with limited data to guide management. This retrospective study documents UK management approaches across 14 centres over 11 years., Materials and Methods: Patients with WHO G3 meningioma between 01/01/2008 and 31/12/2018 were identified. Data were collected on demographics, management strategy, adjuvant radiotherapy, approach in recurrence setting and survival., Results: 84 patients were identified. 21.4% transformed from lower-grade disease. 96.4% underwent primary surgical resection, with 20.8% having evidence of residual disease on their post-op MRI. 59.3% of patients underwent adjuvant radiotherapy (RT) following surgical resection. Overall median PFS and OS were 12.6 months and 28.2 months, respectively. Median OS in the group who underwent complete surgical resection was 34.9 months, compared to 27.5 months for those who had incomplete resection (HR 0.58, 95% CI 0.27-1.23, p = 0.15). Median OS was 33.1 months for those who underwent adjuvant RT and 14.0 months for those who did not (HR 0.48, 95% CI 0.27-0.84, p = 0.004). Median adjuvant RT dose delivered was 60Gy (range 12Gy-60Gy), 45.8% of adjuvant RT was delivered using IMRT. At disease relapse, 31% underwent salvage surgery and 29.3% underwent salvage RT. Of those treated with salvage RT, 64.7% were re-treats and all were treated with hypofractionated RT., Conclusion: Surgery continues to be the preferred primary management strategy. Post-operative MRI within 48 hours is indicated to assess presence of residual disease and guide further surgical options. Adjuvant radiotherapy plays an important part of the management paradigm in these patients with the data supporting an attached survival advantage. Further surgery and re-irradiation is an option in the disease recurrence setting with radiosurgery frequently utilised in this context., (Crown Copyright © 2024. Published by Elsevier Ltd. All rights reserved.)

Background: The Simpson grading scale assumes dural resection (grade I) is more effective against recurrence than coagulation (grade II). However, the results of recent studies have raised doubts about this effectiveness in spinal meningiomas. Therefore, we aimed to perform a meta-analysis comparing outcomes between Simpson grades I and II in spinal meningiomas., Methods: According to the PRISMA statement, we systematically searched PubMed, EMBASE, and Web of Science for studies involving patients with spinal meningiomas who underwent Simpson grades I, II, III, or IV. Outcomes were radiological tumor recurrence, postoperative neurological deficits, and procedure-related complications., Results: We included 54 studies with a total of 3334 patients. Simpson grades I, II, III, and IV were performed in 674 (20%), 2205 (66%), 254 (8%), and 201 (6%) patients, respectively. The follow-up ranged from 9 to 192 months, and 95.4% of all tumors were WHO grade 1. There was no difference in radiological tumor recurrence (OR 0.80, 95% CI: 0.46-1.36, P = 0.41; I 2  = 0%), postoperative neurological deficits (OR 0.74, 95% CI: 0.32-1.75, P = 0.50; I 2  = 0%) or procedure-related complications (OR 2.22, 95% CI: 0.80-6.13, P = 0.12; I 2  = 3%) between Simpson grades I and II. Furthermore, no significant difference in postoperative neurological deficits or procedure-related complications was detected when comparing all Simpson's to each other. However, radiological tumor recurrences in Simpson I and II were significantly lower than in III and IV, with Simpson III outperforming IV (OR 0.19, 95% CI: 0.09-0.40, P < 0.01; I 2  = 0%)., Conclusion: Simpson grade I is not more effective than grade II in any outcome, although both are superior to III and IV in tumor recurrence. Our results might suggest that dural coagulation is preferable over resection when the latter carries a higher risk of complications., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Austria, part of Springer Nature.)

Background: Our purpose was to determine the utility of [68Ga]-DOTATATE PET/MRI in meningioma response assessment following radiosurgery., Methods: Patients with meningioma prospectively underwent postoperative DOTATATE PET/MRI. Co-registered PET and gadolinium-enhanced T1-weighted MRI were employed for radiosurgery planning. Follow-up DOTATATE PET/MRI was performed at 6-12 months post-radiosurgery. Maximum absolute standardized uptake value (SUV) and SUV ratio (SUVRSSS) referencing superior sagittal sinus (SSS) blood pool were obtained. Size change was determined by Response Assessment in Neuro-Oncology (RANO) criteria. Association of SUVRSSS change magnitude and progression-free survival (PFS) was evaluated using Cox regression., Results: Twenty-seven patients with 64 tumors (26% World Health Organization [WHO]-1, 41% WHO-2, 26% WHO-3, and 7% WHO-unknown) were prospectively followed post stereotactic radiosurgery (SRS) or stereotactic body radiotherapy (SBRT; mean dose: 30 Gy, modal dose 35 Gy, mean of 5 fractions). Post-irradiation SUV and SUVRSSS decreased by 37.4% and 44.4%, respectively (P < .0001). Size product decreased by 8.9%, thus failing to reach the 25% significance threshold as determined by RANO guidelines. Mean follow-up time was 26 months (range: 6-44). Overall mean PFS was 83% and 100%/100%/54% in WHO-1/-2/-3 subcohorts, respectively, at 34 months. At maximum follow-up (42-44 months), PFS was 100%/83%/54% in WHO-1/-2/-3 subcohorts, respectively. Cox regression analyses revealed a hazard ratio of 0.48 for 10-unit reduction in SUVRSSS in the SRS cohort., Conclusions: DOTATATE PET SUV and SUVRSSS demonstrated marked, significant decrease post-radiosurgery. Lesion size decrease was statistically significant; however, it was not clinically significant by RANO criteria. DOTATATE PET/MR thus represents a promising imaging biomarker for response assessment in meningiomas treated with radiosurgery., Clinicaltrials.gov Identifier: NCT04081701., (© The Author(s) 2024. Published by Oxford University Press on behalf of the Society for Neuro-Oncology. All rights reserved. For commercial re-use, please contact reprints@oup.com for reprints and translation rights for reprints. All other permissions can be obtained through our RightsLink service via the Permissions link on the article page on our site—for further information please contact journals.permissions@oup.com.)

Among autophagic-related proteins, p62/SQSTM1/Sequestosome-1 represents a relevant actor in cellular proliferation and neoplastic growth. Although, recently, p62 expression has been analyzed in different neurodegenerative and glial neoplastic diseases, no available information have been reported in meningiomas, which have an high epidemiological relevance being the second most common category of intracranial tumors after gliomas. Generally meningiomas have a benign behavior, but their recurrence is not uncommon mainly when atypical or anaplastic varieties occur. However, intranuclear vacuoles have been ultrastructurally observed in meningiomas, and they were labelled by p62 antibodies. Therefore, in the present study, we have investigated p62 immunohistochemical pattern in a cohort of 133 cases representative of low- and high-grade meningiomas, to verify if p62 expression may be related to clinicopathological data, thus achieving a potential prognostic role. The p62 immunoexpression was frequently found in the nucleus and cytoplasm of neoplastic elements, and utilizing an intensity-distribution score, 55 (41.3%) cases were considered as high expressors while 78 (58.7%) cases were instead recorded as low expressors. Fifteen cases exhibited recurrences of the disease, 14 of which were codified as high expressors. Moreover, a direct relationship between p62 and Mib-1 immunoexpression as well as between p62 and neoplastic grade have been documented. Finally, we suggest that impaired autophagic flux with an increase in p62 expression may be involved in the activation of NRF2 also contributing in the development of recurrence in meningioma patients., Competing Interests: The authors declare that they have no conflicts of interest., (Copyright © 2024 Antonio Ieni et al.)

Background: A primary pulmonary meningioma is an extremely rare entity. Primary pulmonary meningiomas manifested with a ground glass nodule are a very rare occurrence in clinical practice., Case Presentation: In this study, we report a case of a primary pulmonary meningioma with atypical computed tomography features. A 59-year-old Han Chinese female came to our hospital for treatment and reported that her physical examination revealed a ground glass nodule in the right lung for over 3 months. The histologic result revealed a primary pulmonary meningioma. The patient underwent a thoracoscopic lung wedge resection of the right upper lobe for a ground glass nodule. After 1 year of follow-up, the patient is still alive without evidence of metastasis or recurrence., Conclusions: Primary pulmonary meningiomas could have a variety of radiological findings. As there are no specific radiologic features for the diagnosis of primary pulmonary meningiomas, complete resection of the lesion is required for both diagnosis and treatment. It is necessary to note the imaging features of primary pulmonary meningiomas, presenting as a ground glass nodule; this rare tumor should be considered in differential diagnoses., (© 2024. The Author(s).)

Purpose: Meningeal solitary fibrous tumour (SFT) and haemangiopericytoma (HPC) are uncommon tumours that have been merged into a single entity in the last 2021 WHO Classification of Tumors of the Central Nervous System. To describe the epidemiology of SFT/HPC operated in France and, to assess their incidence., Methods: We processed the French Brain Tumour Database (FBTDB) to conduct a nationwide population-based study of all histopathologically confirmed SFT/HPC between 2006 and 2015., Results: Our study included 399 SFT/HPC patients, operated in France between 2006 and 2015, in one of the 46 participating neurosurgical centres. The incidence reached 0.062, 95% CI[0.056-0.068] for 100,000 person-years. SFT accounted for 35.8% and, HPC for 64.2%. The ratio of SFT/HPC over meningioma operated during the same period was 0.013. SFT/HPC are about equally distributed in women and men (55.9% vs. 44.1%). For the whole population, mean age at surgery was 53.9 (SD ± 15.8) years. The incidence of SFT/HPC surgery increases with the age and, is maximal for the 50-55 years category. Benign SFT/HPC accounted for 65.16%, SFT/HPC of uncertain behaviour for 11.53% and malignant ones for 23.31%. The number of resection progresses as the histopathological behaviour became more aggressive. 6.7% of the patients with a benign SFT/HPC had a second surgery vs.16.6% in case of uncertain behaviour and, 28.4% for malignant SFT/HPC patients., Conclusion: Meningeal SFT and HPC are rare CNS mesenchymal tumours which both share common epidemiological characteristics, asserting their merging under a common entity. SFT/HPC incidence is less that one case for 1 billion per year and, for around 100 meningiomas-like tumours removed, one SFT/HPC may be diagnosed. SFT/HPC are equally distributed in women and men and, are mainly diagnosed around 50-55 years. The more aggressive the tumour, the higher the probability of recurrence., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Austria, part of Springer Nature.)

Objective: To analyze the association between fibulin-2 and Ki-67 index with histopathological grade and other clinicopathological factors in patients with meningioma among the Minangkabau ethnic group., Methods: This cross-sectional observational study uses 50 specimens comprising 25 low-risk meningioma cases and 25 high-risk meningioma cases obtained at three anatomical pathology laboratories in Padang, West Sumatra, Indonesia, between 2019 and 2022. All samples were stained using an immunohistochemistry procedure with Ki-67 and fibulin-2. The chi-square test was used to assess IBM SPSS statistics version 26 for Windows was used to assess the association of Ki-67 and fibulin-2 with the histopathological grade. The p-value of <0.05 was considered significant., Result: We found a significant association between Ki-67 (p = 0.013) or fibulin-2 (p = 0.001) expression with histopathological grade of meningioma. High histopathological grade has high expression of Ki-67 and fibulin-2, with Odds ratio (OR) of 13.500 (1.556-117.137) and 10.028 (2,738-36,722), respectively. Fibulin-2 expression was also associated with the age of patients (p = 0.020). The low age group (<50) has high expression of fibulin-2 (OR 0.196 (0.056-0.691).  Conclusion: Ki-67 and fibulin-2 were associated with the histopathological grade of meningioma, while fibulin-2 is also associated with age in the Minangkabau ethnic group.

Background: Spinal intradural tumors are rare and heterogeneous in histological type, aggressiveness, and symptomatology, and there is a lack of data about them. This study investigated the epidemiological features of spinal intradural tumors., Methods: This retrospective analysis included patients with spinal intradural tumors who underwent surgical treatment at the Myelopathy and Spondylosis Ward Beijing Jishuitan Hospital between January 2012 and December 2022., Results: This study included 1321 patients [aged 47.19 ± 14.90 years, 603 (45.65%) males] with spinal intradural tumors. The most common histological subtype was schwannoma [n = 511 (38.68%)], followed by spinal meningioma [n = 184 (13.93%)] and ependymoma [n = 101 (7.65%)]. Fifteen (1.14%) patients were diagnosed with metastatic spinal intradural tumors as a presentation of another primary cancer type. The spinal intradural tumors were mostly found in the lumbar region [n = 436 (33.01%)], followed by the thoracic vertebrae [n = 390 (29.52%)], cervical vertebrae [n = 154 (11.66%)], and thoracolumbar region [n = 111 (8.40%)]. Schwannomas mostly affected the lumbar region [n = 256 (52.64%)], spinal meningiomas in the thoracic region [n = 153 (83.15)], and ependymomas in the lumbar region [56 (55.45%)]. The de novo metastases were mostly found in the lumbar region [n = 8 (53.33%)]., Conclusion: According to the results of our single-center study, the most common spinal intradural tumor in Northern China is schwannoma, followed by spinal meningioma and ependymoma., (© 2024. The Author(s).)

Background: Intracranial solitary fibrous tumors (SFTs) are rare mesenchymal neoplasms, often challenging to diagnose due to their resemblance to meningiomas and other central nervous system tumors. While advancements in molecular genetics have aided in classification, diagnostic nuances and optimal management strategies remain areas of interest., Materials and Methods: This retrospective study analyzed 11 cases of intracranial SFTs treated at a neurosurgical centre in India between February 2020 and January 2024. Clinical data, radiological findings, histopathological features, and follow-up details were reviewed. Immunohistochemistry, particularly STAT6, facilitated diagnosis confirmation., Results: The median age of presentation was 32 years, with a male predominance. Headache was the most common presenting symptom, often leading to misdiagnosis as meningiomas on radiological imaging. Histologically, SFTs exhibited spindle to ovoid cells with staghorn vessels and collagenized stroma, posing challenges in differential diagnosis. WHO grading predominantly revealed grade 1 tumors, though recurrence occurred, emphasizing the importance of long-term follow-up. Immunohistochemistry, particularly STAT6, played a pivotal role in distinguishing SFTs from other entities., Conclusion: Intracranial SFTs present diagnostic challenges due to overlapping features with other tumors, warranting a comprehensive approach integrating clinical, radiological, and histopathological findings. Immunohistochemistry, particularly STAT6, emerges as a valuable diagnostic tool. Long-term follow-up is essential for monitoring recurrence and potential malignant transformation. Further research is needed to delineate optimal treatment strategies, including the role of radiotherapy in SFT management., Competing Interests: Declaration of Competing Interest The authors have no conflicts of interest to declare., (Copyright © 2024 Elsevier GmbH. All rights reserved.)

Background: Primary intraosseous meningioma of the skull (PIMS) is a rare type of primary extradural meningioma (PEM) involving cranial bone. The existing literature strongly suggest the importance of radiological feacures in pathological diagnosis of PIMS. Thereby, the aim of this study is to investigate the association between imaging classification and histopathological grading in PIMS., Methods: In this retrospective study, we retrospectively analyzed the computed tomography scan/magnetic resonance imaging and pathological data pertaining to patients with pathologically proven PIMS. The association between radiological features, imaging classification, and histopathological grading was analyzed using logistic regression analysis., Results: In this study, data of 25 patients with PIMS were assessed. The univariate logistic regression analysis results showed significant correlation between histopathological grading and imaging classification (OR: 22.5; 95% CI: 2.552-198.378; p = 0.005), intra- and extracalvarial extension (OR: 7.2; 95% CI: 1.066-48.639; p = 0.043), and tumor margin (OR: 7.19; 95% CI: 1.06-47.61; p = 0.043). According to the results of multivariate logistic regression analysis, imaging classification was the strongest independent risk factor for high-grade PIMS, and the risk of aggressiveness of osteoblastic type of PIMS was 16.664 times higher than that of osteolytic type of PIMS (OR: 16.664; 95% CI: 1.15-241.508; p = 0.039)., Conclusions: Imaging classification is an independent risk factor for high-grade PIMS., Competing Interests: Declaration of Competing Interest The authors declare that they have no competing interests., (Copyright © 2024. Published by Elsevier B.V.)

Purpose: To examine demographic and clinical characteristics and their association with survival in grade 2 and 3 pediatric meningiomas in a large cohort using the National Cancer Database (NCDB)., Methods: We conducted a comprehensive analysis using data from NCDB between 2004 to 2018. Tumor-specific data included tumor grade and size. Treatment details, including surgical resection, extent of resection, and radiotherapy, were gathered. Our analytic approach incorporated logistic and Poisson regression, Kaplan-Meier survival estimates, and Cox proportional hazards models., Results: Among the included 239 patients aged 0-21 years, age category distribution was significantly different between grade 2 and grade 3 tumors (p = 0.018). For grade 2 meningiomas, 51.5% of patients were female, and 76.7% were white. 85.3% of patients with grade 2 meningiomas underwent surgical resection, of which 67% underwent gross total resection. Overall survival (OS) was significantly different between resected and non-resected patients (p = 0.048). Uninsured patients were over seven times as likely to have prolonged length of stay (LOS) versus those with private insurance (OR = 7.663, p = 0.014). For grade 3 meningiomas, 51.4% of patients were male, and 82.9% were white. 91.4% of patients with grade 3 meningiomas underwent surgical resection, of which 53.3% underwent subtotal resection. OS was not significantly different between resected and non-resected patients (p = 0.659)., Conclusion: In summary, there were significant differences in age, maximum tumor dimension, unplanned readmission, radiotherapy, and treatment combinations between grade 2 and 3 meningiomas. These findings highlight the intricacies of managing pediatric meningiomas and emphasize the necessity for tailored therapeutic approaches to enhance outcomes in the future., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: Meningioma is the most common intracranial tumor, graded on pathology using WHO criteria to predict tumor course and treatment. However, pathological grading via biopsy may not be possible in cases with poor surgical access due to tumor location. Therefore, our systematic review aims to evaluate whether diagnostic imaging features can differentiate high grade (HG) from low grade (LG) meningiomas as an alternative to pathological grading., Methods: Three databases were searched for primary studies that either use routine magnetic resonance imaging (MRI) or computed tomography (CT) to assess pathologically WHO-graded meningiomas. Two investigators independently screened and extracted data from included studies., Results: 24 studies met our inclusion criteria with 12 significant (p < 0.05) CT and MRI features identified for differentiating HG from LG meningiomas. Cystic changes in the tumor had the highest specificity (93.4%) and irregular tumor-brain interface had the highest positive predictive value (65.0%). Mass effect had the highest sensitivity (81.0%) and negative predictive value (90.7%) of all imaging features. Imaging feature with the highest accuracy for identifying HG disease was irregular tumor-brain interface (79.7%). Irregular tumor-brain interface and heterogenous tumor enhancement had the highest AUC values of 0.788 and 0.703, respectively., Conclusion: Our systematic review highlight imaging features that can help differentiate HG from LG meningiomas., (© 2024. The Author(s).)

A meningioma is a common primary central nervous system tumor. The histological features of meningiomas vary significantly depending on the grade and subtype, leading to differences in treatment and prognosis. Therefore, early diagnosis, grading, and typing of meningiomas are crucial for developing comprehensive and individualized diagnosis and treatment plans. The advancement of artificial intelligence (AI) in medical imaging, particularly radiomics and deep learning (DL), has contributed to the increasing research on meningioma grading and classification. These techniques are fast and accurate, involve fully automated learning, are non-invasive and objective, enable the efficient and non-invasive prediction of meningioma grades and classifications, and provide valuable assistance in clinical treatment and prognosis. This article provides a summary and analysis of the research progress in radiomics and DL for meningioma grading and classification. It also highlights the existing research findings, limitations, and suggestions for future improvement, aiming to facilitate the future application of AI in the diagnosis and treatment of meningioma., Competing Interests: Declaration of Competing Interest The authors declare the following financial interests/personal relationships which may be considered as potential competing interests: Zhang Jing reports financial support was provided by Fifth Affiliated Hospital of Zunyi Medical University. Zhang Jing reports a relationship with Fifth Affiliated Hospital of Zunyi Medical University that includes: employment. If there are other authors, they declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 The Association of University Radiologists. Published by Elsevier Inc. All rights reserved.)

Meningioma is the most common type of primary brain tumor. Surgical resection followed by surveillance is the first-line treatment for the majority of symptomatic meningiomas; however, recent advances in molecular sequencing, DNA methylation, proteomics, and single-cell sequencing provide insights into further characterizing this heterogeneous group of tumors with a wide range of prognoses. A subset of these tumors are highly aggressive and cause severe morbidity and mortality. Therefore, identifying those individuals with a poor prognosis and intervening are critical. This review aims to help readers interpret the molecular profiling of meningiomas to identify patients with worse prognoses and guide the management and strategy for surveillance., (© 2024 American Cancer Society.)

Background and Objectives: Recent molecular analyses have shown that the driver genetic mutations of meningiomas were associated with the anatomic location. Among these, POLR2A mutation is common among lesions in the skull base, mainly in the cerebellopontine angle (CPA). The objective of this study was to investigate the efficacy of POLR2A mutation as a prognostic marker for CPA meningiomas., Methods: We retrospectively analyzed the clinical data of 70 patients who had World Health Organization grade I CPA meningiomas. Somatic DNA was analyzed by Sanger sequencing and microsatellite array to examine for NF2 , AKT1 , KLF4 , SMO , and POLR2A mutations and 22q loss. Genetic and clinical parameters were analyzed to identify the factors related with tumor recurrence., Results: We detected clearly the clinical features of the CPA cases with POLR2A mutation. Compared with cases without POLR2A mutation, cases with POLR2A mutation had more meningothelial type ( P = 6.9 × 10 -4 ), and higher rate of recurrence ( P = .04). We found that the poor prognostic factors associated with the recurrence of CPA meningiomas were POLR2A mutation ( P = .03, hazard ratio [HR] 9.38, 95% CI 1.26-70.0) and subtotal resection (STR) ( P = 5.1 × 10 -4 , HR 63.1, 95% CI 6.09-655.0). In addition, in the group that underwent STR, POLR2A mutation was a poor prognostic factor associated with tumor recurrence ( P = .03, HR 11.1, 95% CI 1.19-103.7)., Conclusion: POLR2A mutation and STR were the poor prognostic markers associated with the recurrence of CPA meningioma. For CPA meningioma cases that underwent STR, only POLR2A mutation was a poor prognostic factor. Detecting POLR2A mutation may be a cost-effective, easy, and useful marker for prognostication., (Copyright © Congress of Neurological Surgeons 2024. All rights reserved.)

Background: Schwannomas and meningiomas are the most common intradural extramedullary spinal tumors; however, differentiating between them using magnetic resonance imaging (MRI) is a frequent challenge. In this study, we aimed to investigate the use of the contrast ratio (CR) as a quantitative MRI method in the differentiation of schwannomas and meningiomas., Methods: We analyzed the data of patients with intradural extramedullary spinal tumors who underwent surgery and were diagnosed with either schwannomas or meningiomas by histopathological analysis. Regions of interest were set for the entire spinal tumor on T2-weighted sagittal MRI. To obtain the CR values of spinal tumors (CR tumor ), we used the signal intensity (SI) values of the tumor (SI tumor ) and spinal cord (SI cord ) according to the following formula: [CR tumor  = (SI tumor -SI cord )/(SI tumor +SI cord )]., Results: The study included 50 patients (23 males and 27 females) with a mean age of 61.5 years old (11-85 years old). Histopathological analysis revealed that 33 and 17 patients were diagnosed with schwannomas and meningiomas, respectively. The mean CR values of the schwannomas and meningiomas were 0.3040 ± 0.1386 and 0.0173 ± 0.1929, respectively. The CR value of the schwannomas was statistically significantly higher than that of meningiomas (P < 0.01). The cutoff CR value obtained from the receiver operating characteristic curve was 0.143, with a specificity and sensitivity of 90.9% and 88.2%, respectively. Furthermore, the value for the area under the receiver operating characteristic curve was 0.925 (95% confidence interval: 0.852-0.998)., Conclusions: The evaluation of CRs by using MRI to distinguish between schwannomas and meningiomas is a beneficial quantitative tool., (Copyright © 2024. Published by Elsevier Inc.)

Meningiomas are the most common primary intracranial tumors and account for nearly 30% of all nervous system tumors. Approximately half of meningioma patients exhibit neurofibromin 2 (NF2) gene inactivation. Here, NF2 was shown to interact with the endoplasmic reticulum (ER) calcium (Ca 2+ ) channel inositol 1,4,5-trisphosphate receptor 1 (IP3R1) in IOMM-Lee, a high-grade malignant meningioma cell line, and the F1 subdomain of NF2 plays a critical role in this interaction. Functional assays indicated that NF2 promotes the phosphorylation of IP3R (Ser 1756) and IP3R-mediated endoplasmic reticulum (ER) Ca 2+ release by binding to IP3R1, which results in Ca 2+ -dependent apoptosis. Knockout of NF2 decreased Ca 2+ release and promoted resistance to apoptosis, which was rescued by wild-type NF2 overexpression but not by F1 subdomain deletion truncation overexpression. The effects of NF2 defects on the development of tumors were further studied in mouse models. The decreased expression level of NF2 caused by NF2 gene knockout or mutation affects the activity of the IP3R channel, which reduces Ca 2+ -dependent apoptosis, thereby promoting the development of tumors. We elucidated the interaction patterns of NF2 and IP3R1, revealed the molecular mechanism through which NF2 regulates IP3R1-mediated Ca 2+ release, and elucidated the new pathogenic mechanism of meningioma-related NF2 variants. Our study broadens the current understanding of the biological function of NF2 and provides ideas for drug screening of NF2-associated meningioma., (© 2024 The Author(s). The FASEB Journal published by Wiley Periodicals LLC on behalf of Federation of American Societies for Experimental Biology.)

BACKGROUND The BrainLab VectorVision neuronavigation system is an image-guided, frameless localization system used intraoperatively, which includes a computer workstation for viewing and analyzing operative microscopic images. This retrospective study aimed to evaluate the use of the BrainLab VectorVision infrared-based neuronavigation imaging system in 80 patients with intracranial meningioma removed surgically between 2013 and 2023. MATERIAL AND METHODS Data were retrospectively collected from 36 patients with convexity meningioma and 44 patients with parasagittal meningioma between 2013 and 2023. The surgical operation of 40 of these patients was performed with the help of neuronavigation, while the other 40 were performed without neuronavigation. Demographic data, preoperative and postoperative radiologic images, craniotomy measurements, surgical complications, and operative times of patients with and without neuronavigation were analyzed. RESULTS Using neuronavigation significantly increased surgery duration (P=0.023). In 6 patients without the use of neuronavigation, the craniotomy had to be enlarged and this resulted in superior sagittal sinus (SSS) damage (P=0.77, P=0.107). Patients for whom neuronavigation was used did not experience any sinus damage and did not require craniotomy enlargement. Postoperative epidural hematoma (EH) developed in 9 patients without navigation, whereas it developed in only 1 patient with navigation (P=0.104). Residual tumors were less common in patients using navigation (P=0.237). CONCLUSIONS The use of neuronavigation allows the incision and craniotomy to be reduced in size. Intraoperatively, it allows the surgeon to master the boundaries of the tumor and surrounding vascular structures, reducing the risk of complications. These results suggest that neuronavigation systems are an effective ancillary in meningioma surgery.

Background: Intraventricular meningioma (IVM) is a rare subtype of intracranial meningioma, accounting for 9.8 to 14% of all intraventricular tumors. Currently, there is no clear consensus on which patients with IVM should receive conservative treatment, surgery, or stereotactic radiosurgery (SRS). This research aims to analyze the outcomes, including survival and recurrence rates of patients who undergo SRS for IVM as a primary or adjuvant treatment., Methods: A systematic search was conducted in Scopus, Web of Science, PubMed, and Embase till June 5th 2023. Screening and data extraction were performed by two independent authors. Random-effect meta-analysis was performed to determine the tumor control proportion of IVM cases treated with SRS. Individual patient data (IPD) meta-analysis was performed for the progression-free survival (PFS) of the patients in the follow-up time. All analyses were performed using the R programming language., Results: Out of the overall 132 records, 14 were included in our study, of which only 7 had enough data for the meta-analysis. The tumor control proportion was 0.92 (95% CI, 0.69-0.98) in patients who underwent SRS for primary IVM. The overall tumor control in both primary and adjuvant cases was 0.87 (95% CI, 0.34-0.99). the heterogeneity was not significant in both meta-analyses (P = 0.73 and P = 0.92, respectively). Post-SRS perifocal edema occurred in 16 out of 71 cases (0.16; 95% CI, 0.03-0.56), with no significant heterogeneity (P = 0.32). IPD meta-analysis showed a PFS of 94.70% in a 2-year follow-up. Log-rank test showed better PFS in primary SRS compared to adjuvant SRS (P < 0.01)., Conclusions: According to this study, patients with IVM can achieve high rates of tumor control with a low risk of complications when treated with SRS, regardless of whether they have received prior treatment. Although SRS could be a promising first-line treatment option for asymptomatic IVM, its efficacy in symptomatic patients and its comparison with resection require further investigation., (© 2024. The Author(s).)

Purpose: We conducted a National Cancer Database (NCDB) study to investigate the epidemiological characteristics and identify predictors of outcomes associated with geriatric meningiomas., Methods: The NCDB was queried for adults aged 60-89 years diagnosed between 2010 and 2017 with grade 2 and 3 meningiomas. The patients were classified into three age groups based on their age: 60-69 (hexagenarians), 70-79 (septuagenarians), and 80-89 (octogenarians). The log-rank test was utilized to compare the differences in overall survival (OS). Univariate and multivariate Cox proportional hazards regressions were used to evaluate the mortality risk associated with various patient and disease parameters., Results: A total of 6585 patients were identified. Hexagenerians were the most common age group (49.8%), with the majority of meningiomas being classified as grade 2 (89.5%). The incidence of high-grade meningiomas increased in all age groups during the study period. Advanced age, male sex, black race, lower socioeconomic status, Charlson-Deyo score ≥ 2, and higher tumor grade were independent factors of poor survival. Among the modes of treatment, the extent of surgical resection, adjuvant radiotherapy, and treatment at a noncommunity cancer program were linked with better outcomes., Conclusion: In geriatric patients with high-grade meningiomas, the greater extent of surgical resection and radiotherapy are associated with improved survival. However, the management and outcome of geriatric patients with higher-grade meningiomas are also associated with several socioeconomic factors., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Austria, part of Springer Nature.)

Background: We previously reported that tumor 3D volume growth rate (3DVGR) classification could help in the assessment of drug activity in patients with meningioma using 3 main classes and a total of 5 subclasses: class 1: decrease; 2: stabilization or severe slowdown; 3: progression. The EORTC-BTG-1320 clinical trial was a randomized phase II trial evaluating the efficacy of trabectedin for recurrent WHO 2 or 3 meningioma. Our objective was to evaluate the discriminative value of 3DVGR classification in the EORTC-BTG-1320., Methods: All patients with at least 1 available MRI before trial inclusion were included. 3D volume was evaluated on consecutive MRI until progression. 2D imaging response was centrally assessed by MRI modified Macdonald criteria. Clinical benefit was defined as neurological or functional status improvement or steroid decrease or discontinuation., Results: Sixteen patients with a median age of 58.5 years were included. Best 3DVGR classes were: 1, 2A, 3A, and 3B in 2 (16.7%), 4 (33.3%), 2 (16.7%), and 4 (33.3%) patients, respectively. All patients with progression-free survival longer than 6 months had best 3DVGR class 1 or 2. 3DVGR classes 1 and 2 (combined) had a median overall survival of 34.7 months versus 7.2 months for class 3 (P = .061). All class 1 patients (2/2), 75% of class 2 patients (3/4), and only 10% of class 3 patients (1/10) had clinical benefit., Conclusions: Tumor 3DVGR classification may be helpful to identify early signals of treatment activity in meningioma clinical trials., (© The Author(s) 2024. Published by Oxford University Press on behalf of the Society for Neuro-Oncology. All rights reserved. For commercial re-use, please contact reprints@oup.com for reprints and translation rights for reprints. All other permissions can be obtained through our RightsLink service via the Permissions link on the article page on our site—for further information please contact journals.permissions@oup.com.)

Background: Meningioma is the most common primary intracranial tumor with a high frequency of postoperative recurrence, yet the biology of the meningioma malignancy process is still obscure., Methods: To identify potential therapeutic targets and tumor suppressors, we performed single-cell transcriptome analysis through meningioma malignancy, which included 18 samples spanning normal meninges, benign and high-grade in situ tumors, and lung metastases, for extensive transcriptome characterization. Tumor suppressor candidate gene and molecular mechanism were functionally validated at the animal model and cellular levels., Results: Comprehensive analysis and validation in mice and clinical cohorts indicated clusterin (CLU) had suppressive function for meningioma tumorigenesis and malignancy by inducing mitochondria damage and triggering type 1 interferon pathway dependent on its secreted isoform, and the inhibition effect was enhanced by TNFα as TNFα also induced type 1 interferon pathway. Meanwhile, both intra- and extracellular CLU overexpression enhanced macrophage polarization towards M1 phenotype and TNFα production, thus promoting tumor killing and phagocytosis., Conclusions: CLU might be a key brake of meningioma malignance by synchronously modulating tumor cells and their microenvironment. Our work provides comprehensive insights into meningioma malignancy and a potential therapeutic strategy., (© The Author(s) 2024. Published by Oxford University Press on behalf of the Society for Neuro-Oncology. All rights reserved. For commercial re-use, please contact reprints@oup.com for reprints and translation rights for reprints. All other permissions can be obtained through our RightsLink service via the Permissions link on the article page on our site—for further information please contact journals.permissions@oup.com.)

Background: Although the imaging findings of intracranial solitary fibrous tumor (SFT)/hemangiopericytoma (HPC) and meningioma are similar, their treatment and prognosis are quite different. Accurate preoperative identification of these two types of tumors is crucial for individualized treatment., Objective: The aim of this study was to develop a radiomics model for the differentiation of intracranial SFT/HPC and meningioma based on multiparametric magnetic resonance imaging (mpMRI)., Material and Methods: A total of 99 patients from July 2012 to July 2018 with histologically and immunohistochemically confirmed SFT/HPC (n = 40) or meningiomas (n = 59) were retrospectively analyzed. A total of 1118 features were extracted based on its image shape, intensity and texture features. The logistic regression (LR) and multi-layer artificial neural network (ANN) classifiers were used to classify SFT/HPC and meningioma. The predictive performance was calculated using receiver operating characteristic curves (ROC)., Results: We found significant difference in terms of sex between the SFT/HPC and meningioma group (χ2= 4.829, P < 0.05), but no significant difference was found in age (P > 0.05). The most significant radiomics features included five shape and four first-order level features. For the LR classifier, the prediction accuracy of SFT/HPC was 71.0% and meningioma was 78.7%. For the ANN classifier, the prediction accuracy of SFT/HPC was 83.9% and meningioma was 80.9%. Both of the two classifiers achieved a high accuracy rate, but ANN was better., Conclusions: Radiomics features, especially when combined with an ANN classifier, can provide satisfactory performance in distinguishing SFT/HPC and meningioma., (Copyright © 2024 Copyright: © 2024 Neurology India, Neurological Society of India.)

The need for resection of the meningioma capsule has long been debated and remains controversial. Even the definition of a capsule does not seem to have been established. We described the histopathological findings of the so-called capsule and the necessity for resection, considering tumor cell invasion and recurrence, based on a literature review.

Most meningiomas are benign, slow growing tumors, which rarely progress to a higher grade. The incidence rate of malignant progression is estimated to be 2.98/1000 patient-year. However, non-skull base location is a significant risk factor for progression. The median time to malignant progression is 4.3 years; however, the cumulative rate of progression approaches a plateau after 10 years. Although radiosurgery does not appear to increase the incidence rate(0.5/1000 patient-year), exact comparisons have been difficult because of differences in study populations. The median time to progression is 7.0 years from initial diagnosis and 5.0 years from radiosurgery. The cumulative rate appears to increase even after 10 years. The risk of malignant transformation after radiotherapy may increase in patients with tumor-prone syndromes, with some controversies regarding patients with neurofibromatosis type 2. Although short term follow-up in patients with meningioma suggests that radiosurgery is safe, there is uncertainty regarding its use in pediatric patients, and those with tumor-prone syndromes.

Meningiomas are the most common brain tumors, often in the form of extra-axial masses adhering to the dura mater. Although there are typical imaging findings, meningiomas have a wide variety of imaging findings, owing to their different histological subtypes. Thus, it can be difficult to differentiate meningiomas from other diseases that present with similar imaging findings. This section outlines mimickers for monitoring meningiomas that present with imaging findings similar to those of meningiomas. Diseases that form masses and require differentiation from meningiomas include schwannomas, solitary fibrous tumors, dural metastases, and histiocytosis. Diseases that primarily present as dural thickening and require differentiation from meningiomas include hypertrophic duralitis, fungal infections, and IG4-related diseases. Notably, in addition to the various pathologies that can mimic meningiomas, such as those listed above, there are also cases in which the diagnosis of meningioma is difficult because of additional modifications, such as metastasis or meningioma infarction.

Meningiomas are the most common primary intracranial tumors. As the number of incidentally discovered meningiomas has increased with the widespread access and use of neuroimaging, treatment strategies for meningiomas have become more important. Close observation is the first choice for asymptomatic lesions; however, the natural history of meningiomas remains unclear. It is necessary to recognize the characteristics of meningiomas that are likely to grow, such as high signal intensity on MRI T2WI. It is also important to examine the growth rates and patterns using multiple neuroimaging examinations during the follow-up period. The authors suggested a relationship between the various observed growth patterns and the length of the follow-up period based on the assumed development of tumor volume. Less than 10% of patients with asymptomatic meningiomas develop symptoms and require treatment. However, it remains unclear which lesions become symptomatic, and further studies are required. Lesions with a sustained growth pattern undergo preventive treatment interventions, but the need for and appropriate timing of these interventions are continuously under debate. Further studies will help elucidate the natural history of meningiomas.

In contrast to other cancer research areas, the development of chemotherapy and radiotherapy for meningiomas has been challenging, lacking standardized criteria for assessing treatment response and progression. Although efforts by RANO have proposed evaluation criteria, a consensus on effective evaluation parameters for meningiomas remains elusive. This paper underscores the importance of establishing efficacy endpoints in clinical trials, compares efficacy assessment in meningioma research with other solid tumor areas, and outlines the challenges ahead in meningioma research. Recent analyses revealed the absence of consensus on efficacy endpoints in meningioma trials, complicating trial design and hindering cross-trial comparisons. The unique anatomical constraints and histological variability of meningiomas pose challenges in determining appropriate efficacy measures. To address these challenges, future research must focus on accumulating prognostic data, standardizing evaluation criteria, and considering multiple endpoints in trial designs. Akin to other cancer areas, investigating targeted therapies and establishing international consensus on efficacy endpoints are crucial steps forward.

The jugular foramen, also known as the foramen magnum, is a highly intricate region of the skull base through which numerous critical blood vessels and nerves traverse. Meningiomas, the most common tumors in neurosurgical pathology, can arise at any location where the meninges are present, posing significant challenges. Meningiomas involving the jugular foramen and sublingual neural tube are particularly notable for their potential to extend from intracranial to extracranial sites, necessitating familiarity with extracranial anatomy, which is not typically encountered in clinical practice. A comprehensive understanding of anatomical characteristics, along with an ample field of view and working space, is crucial for handling the cerebellum, brainstem, and nerves meticulously. The use of surgical support tools such as neuromonitoring and navigation is essential for enhancing the safety of the procedure. Furthermore, preparedness for treatment options, rehabilitation, and adjunctive therapies is vital in the event of neurological symptoms such as those affecting the glossopharyngeal, vagal, or hypoglossal nerves.

The frequency of identification of asymptomatic meningiomas is increasing owing to the advancement and widespread use of CT and MRI. The first choice for asymptomatic meningiomas is observation. Approximately 70% of asymptomatic meningiomas increase in volume on long term follow-up. More than half of them reportedly exhibit a self-limiting pattern, in which growth eventually stops. Imaging findings related to increased meningioma volume include no calcification, large tumor size, high signal intensity inside the tumor on T2-weighted images, high brightness within the tumor on diffusion-weighted images, and perifocal edema. We also highlighted the presence of hypo-intensity of the surface layer on T2-weighted imaging, indicating growth arrest in a self-limiting growth pattern of meningioma. In this article, literature reports on image prediction using CT and MRI regarding the growth of asymptomatic meningiomas were reviewed, along with our report.

Meningiomas, renowned for their histological diversity, are one of the most prevalent brain tumors. Some meningiomas show unusual histomorphology, especially in intraoperative rapid diagnosis. Therefore, clinical and radiological information is crucial for pathological diagnosis. Before the 2021 World Health Organization Classification of Tumors of the Central Nervous System(5th edition), pathological diagnosis relied solely on histopathological features. However, this classification introduced new diagnostic criteria for anaplastic meningiomas, which now include TERT promoter mutations and the homozygous deletion of CDKN2A/B , indicating the necessity of genetic analysis. Some rhabdoid and papillary meningiomas have BAP1 alterations, which tend to demonstrate an aggressive clinical course and may represent a phenotype of BAP1 -related tumor predisposition syndrome. Heterozygous deletion of CDKN2A/B and loss of H3 p.K28me3(K27me3)are also associated with poor prognosis. Although some immunohistochemical markers like MTAP may serve as surrogates for the homozygous deletion of CKKN2A/B , genetic analysis is required to confirm TERT promoter mutations. Therefore, in routine clinical practice, neurosurgeons and pathologists prioritize appropriate formalin fixation to facilitate genetic analysis using pathological specimens.

Abstract: Neuroblastomas are the most common extracranial solid tumor in the pediatric age group (~8%-10% of childhood neoplasms). Most cases of intracranial neuroblastomas occur due to metastasis from some primary extracranial sites and are known as secondary neuroblastomas. However, the occurrence of primary central nervous system neuroblastomas (PCN-NB) is very rare, and only a few cases and case series have been reported in the literature. PCN-NB is mainly an intra-axial pathology, and extra-axial involvement is mainly due to metastasis from some extracranial primary site with involvement of the skull bone. Herein we report a case of a 23-year-old female having a large extra-axial space-occupying lesion in the right frontal region that was mimicking a meningioma, and surprisingly the histopathology was suggestive of a supratentorial neuroblastoma. A right frontal craniotomy was made, and Simpson's grade 1 excision of the tumor was done. The excised tissue was sent for histopathological examination. PCN-NB located extra-axially are extremely rare to occur. Due to inconsistent radiological imaging, it becomes very difficult to diagnose these tumors preoperatively, and these should be kept in mind as one of the differential diagnoses of extra-axial intracranial space-occupying lesions. Histopathological examination is crucial in diagnosing the intracranial neuroblastomas., (Copyright © 2024 Copyright: © 2024 Journal of Postgraduate Medicine.)

Abstract: Chordoid meningiomas, rare meningioma variants, are characterized by their histopathological features and clinical behavior resembling that of other chondroid/myxoid neoplasms. We present a case of pathology-proven chordoid meningioma imaged with both 68 Ga-DOTATATE and 18 F-FDG PET images during a complicated postoperative course with multiple episodes of local recurrence and, ultimately, extracranial metastasis. This case underscores the aggressive behavior of chordoid meningiomas while highlighting how molecular imaging plays an important role in clinical monitoring and guidance of management., Competing Interests: Conflicts of interest and sources of funding: none declared., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Purpose: Multiple endocrine neoplasia type 1 (MEN1) is thought to increase the risk of meningioma and ependymoma. Thus, we aimed to describe the frequency, incidence, and specific clinical and histological features of central nervous system (CNS) tumors in the MEN1 population (except pituitary tumors)., Experimental Design: The study population included patients harboring CNS tumors diagnosed with MEN1 syndrome after 1990 and followed up in the French MEN1 national cohort. The standardized incidence ratio (SIR) was calculated based on the French Gironde CNS Tumor Registry. Genomic analyses were performed on somatic DNA from seven CNS tumors, including meningiomas and ependymomas from patients with MEN1, and then on 50 sporadic meningiomas and ependymomas., Results: A total of 29 CNS tumors were found among the 1,498 symptomatic patients (2%; incidence = 47.4/100,000 person-years; SIR = 4.5), including 12 meningiomas (0.8%; incidence = 16.2/100,000; SIR = 2.5), 8 ependymomas (0.5%; incidence = 10.8/100,000; SIR = 17.6), 5 astrocytomas (0.3%; incidence = 6.7/100,000; SIR = 5.8), and 4 schwannomas (0.3%; incidence = 5.4/100,000; SIR = 12.7). Meningiomas in patients with MEN1 were benign, mostly meningothelial, with 11 years earlier onset compared with the sporadic population and an F/M ratio of 1/1. Spinal and cranial ependymomas were mostly classified as World Health Organization grade 2. A biallelic MEN1 inactivation was observed in 4/5 ependymomas and 1/2 meningiomas from patients with MEN1, whereas MEN1 deletion in one allele was present in 3/41 and 0/9 sporadic meningiomas and ependymomas, respectively., Conclusions: The incidence of each CNS tumor was higher in the MEN1 population than in the French general population. Meningiomas and ependymomas should be considered part of the MEN1 syndrome, but somatic molecular data are missing to conclude for astrocytomas and schwannomas., (©2024 American Association for Cancer Research.)

Background: External radiation therapy (RT) is often a primary treatment for inoperable meningiomas in the absence of established chemotherapy. Histone deacetylase 6 (HDAC6) overexpression, commonly found in cancer, is acknowledged as a driver of cellular growth, and inhibiting HDACs holds promise in improving radiotherapeutic efficacy. Downregulation of HDAC6 facilitates the degradation of β-catenin. This protein is a key element in the Wnt/β-catenin signalling pathway, contributing to the progression of meningiomas., Methods: In order to elucidate the associations and therapeutic potential of HDAC6 inhibitors (HDAC6i) in conjunction with RT, we administered Cay10603, HDAC6i, to both immortalised and patient-derived meningioma cells prior to RT in this study., Findings: Our findings reveal an increase in HDAC6 expression following exposure to RT, which is effectively mitigated with pre-treated Cay10603. The combination of Cay10603 with RT resulted in a synergistic augmentation of cytotoxic effects, as demonstrated through a range of functional assays conducted in both 2D as well as 3D settings; the latter containing syngeneic tumour microenvironment (TME). Radiation-induced DNA damage was augmented by pre-treatment with Cay10603, concomitant with the inhibition of β-catenin and minichromosome maintenance complex component 2 (MCM2) accumulation within the nucleus. This subsequently inhibited c-myc oncogene expression., Interpretation: Our findings demonstrate the therapeutic potential of Cay10603 to improve the radiosensitisation and provide rationale for combining HDAC6i with RT for the treatment of meningioma., Funding: This work was funded by Brain Tumour Research Centre of Excellence award to C Oliver Hanemann., Competing Interests: Declaration of interests COH received consultating fees from Recursion in 2022. COH is member of ICOM., (Copyright © 2024 The Author(s). Published by Elsevier B.V. All rights reserved.)

Background: Meningiomas are the most frequent primary intracranial tumor. While histological grade and grade of excision are established predictors of recurrence, the predictive ability of other clinical features, such as the role of radical excision of dural attachment and postoperative radiation therapy in intermediate-risk groups, remains unknown., Methods: Clinical and radiological features and surgical details were analyzed in 451 World Health Organization (WHO) grade 1 intracranial meningiomas and 248 WHO grade 2 meningiomas operated on between 2010 and 2015. Outcomes were assessed in 352 WHO grade 1 and 208 WHO grade 2 meningiomas, studying the effect of extent of resection and use of radiation therapy. Kaplan-Meier analysis was used to determine differences in survival by extent of resection and use of postoperative radiation therapy in the treatment of the meningiomas., Results: The mean age of the cohort was 46.3 years, with a female predominance. On univariate analysis, sex, WHO grade, and Simpson grade were significant predictors of recurrence. On multivariate analysis, WHO grade and Simpson grade remained significant predictors of recurrence. Recurrence was significantly associated with poor performance status and mortality. Postoperative radiation significantly improved progression-free survival among patients with grade 2 meningiomas who underwent gross total resection, but not among patients with grade 1 and grade 2 meningiomas who underwent subtotal resection., Conclusions: WHO grade and Simpson grade are independent predictors of recurrence in meningiomas. Regardless of WHO grade, gross total resection must be performed when possible, and postoperative radiation therapy may be recommended in grade 2 meningiomas., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Introduction: Meningiomas are the most common primary brain and central nervous system tumors, accounting for approximately 40% of these tumors. The most important exams for the radiological study of meningiomas are computed tomography (CT) and magnetic resonance imaging (MRI). We aimed to analyze the radiological features of patients with meningioma related to the simultaneous presence of bilateral macronodular adrenocortical disease (BMAD), with or without pathogenic variants of ARMC5., Methods: This study included 10 patients who were diagnosed with BMAD. All of them had a radiological diagnosis of expansive brain lesions suggestive of meningioma. All patients underwent brain MRI and a neuroradiolgist analyzed the following parameters: number, site and size of lesions; presence of calcification, edema and bone involvement., Results and Discussion: Eight patients presented with germline variants of ARMC5; the other 2, did not. The most significant result was the incidence of multiple meningiomas, which was 50% in BMAD patients, whereas the average incidence described thus far is lower than 10%. Considering location, the 22 tumors in the BMAD patients were 5 convexity tumors (22.7%), and 17 skull base tumors (77.2%), the opposite proportion of patients without BMAD. A total of 40.9% of the tumors had calcification, 9% had cerebral edema and 40.9% had bone invasion due to hyperostosis. The literature describes meningioma calcification in 25% of patients, bone invasion by tumor hyperostosis in 20%, and cerebral edema in approximately 60%., Conclusion: Relevant results were found considering the rate of multiple meningiomas and tumor location. This finding reinforces the need for further research into the neurological effects caused by genetic variants of ARMC5 in patients with BMAD., (© 2024. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)