Descriptive epidemiology of 399 histologically confirmed newly diagnosed meningeal solitary fibrous tumours and haemangiopericytomas in France: 2006-2015.

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.

Primary pulmonary meningioma presenting as a pulmonary ground glass nodule: a case report and review of the literature.

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.

Epidemiological features of spinal intradural tumors, a single-center clinical study in Beijing, China.

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.

Immunohistochemical Profile of p62/SQSTM1/Sequestosome-1 in Human Low- and High-Grade Intracranial Meningiomas.

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.

Resection vs. coagulation of dural attachment in patients with spinal meningioma: an updated systematic review and meta-analysis.

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; I2 = 0%), postoperative neurological deficits (OR 0.74, 95% CI: 0.32-1.75, P = 0.50; I2 = 0%) or procedure-related complications (OR 2.22, 95% CI: 0.80-6.13, P = 0.12; I2 = 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; I2 = 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.

NF2 regulates IP3R-mediated Ca2+ signal and apoptosis in meningiomas.

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 (Ca2+) 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) Ca2+ release by binding to IP3R1, which results in Ca2+-dependent apoptosis. Knockout of NF2 decreased Ca2+ 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 Ca2+-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 Ca2+ 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.

[Epidemiology, Natural History, and Growth Risk of Meningiomas].

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.

[Malignant Transformation of Benign Meningiomas with or without Radiotherapy].

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.

[Necessity of Meningioma Capsulectomy].

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.

[Prediction of the Natural Course of Meningioma Using CT and MR Images].

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.

[Pathological Diagnosis and Genetic Alterations of Meningioma].

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.

[Imaging Features and Differential Diagnosis of Meningiomas and Their Mimics].

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.

[Jugular Foramen, Foramen Magnum Meningiomas].

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.

[Challenges to Overcome in Clinical Research on 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.

Clinicopathological characteristics of secondary trigeminal neuralgia due to cerebellopontine angle tumors. / æ¡¥å°è„‘è§’è‚¿ç˜¤ç»§å‘æ€§ä¸‰å‰ç¥žç»ç—›çš„ä¸´åºŠç— ç†ç‰¹å¾.

OBJECTIVES: Cerebellopontine angle (CPA) tumors are a common cause of secondary trigeminal neuralgia (TN), characterized by their concealed location, slow progression, and difficulty in early detection. This study aims to explore the clinicopathological characteristics of patients with secondary TN due to CPA tumors to enhance understanding and management of secondary TN. METHODS: A retrospective analysis was conducted on clinical data and pathological results of 116 patients with CPA tumor-related TN treated at Xiangya Hospital of Central South University from January 1, 2017 to December 31, 2022. The study analyzed the relationship of tumor pathological types with clinical manifestations, tumor location, surgical methods, and treatment outcomes. RESULTS: Among the cases, 95.7% (111/116) were benign tumors, 3.4% (4/116) were malignant tumors, and 0.9% (1/116) were borderline tumors. Benign tumors were predominantly acoustic neuromas, meningiomas, and schwannomas. Among the patients, 46.6% (54/116) presented with isolated TN, while 53.4% (62/116) exhibited other associated symptoms depending on factors such as tumor growth location and rate. The complete resection rate in this group was over 90%, with 41.4% (48/116) of patients undergoing concurrent microvascular decompression after tumor resection, predominantly for schwannomas. The overall effective rate of surgical treatment reached 93.9%, with schwannomas showing higher efficacy rates compared with acoustic neuromas and meningiomas (P<0.05). The recurrence rate of acoustic neuromas was significantly higher than that of meningiomas and schwannomas (P<0.05). CONCLUSIONS: CPA tumors are a major cause of secondary TN, predominantly benign, with occasional underdiagnosed malignant tumors. Early diagnosis and treatment significantly impact prognosis. Different tumor types vary in clinical symptoms, surgical approaches, and treatment efficacy. Surgical strategies should balance tumor resection extent and neural function preservation, with microvascular decompression as necessary.

Atypical and anaplastic meningiomas in the later decades of life: A national cancer database analysis.

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.

Full Endoscopic Transcranial Resection of Meningiomas.

OBJECTIVE: Tumors of the skull base can be accessed through different routes. Recent advantages in minimally invasive techniques have shown that very different routes can be applied for optimal tumor resection depending on the technical equipment, the surgeon's preference, and the individual anatomy of the pathology. Here, the authors present their technique for pure endoscopic transcranial tumor resection in meningiomas. METHODS: Out of the cases of the Department of Neurosurgery, Homburg Saar Germany of the last 10 years, all endoscopic procedures for meningiomas were analyzed. Particular attention was given to evaluating the peculiarities of those meningiomas that were treated purely endoscopically. RESULTS: While the endoscope was used in a large number of skull base meningiomas in endonasal approaches or for endoscopic inspection in transcranial skull base surgery, only a small number of meningiomas was selected for a purely endoscopically performed resection. The characteristics of these cases were rather a small lesion, straight access, and a keyhole position of the craniotomy. A complete resection of the tumor was achieved in all cases. Conversion to the microscope was not necessary in any case. There were no technical issues or complications associated with a fully endoscopic resection. DISCUSSION: The endoscope is a valuable tool for visualization in meningioma surgery. In most cases, it is applied for an endonasal route or for endoscopic inspection in transcranial microsurgical cases. However, small to medium-sized meningiomas that can be accessed through the keyhole approach are good candidates for pure endoscopic resection. Because of the very high magnification and the minimally invasive nature of this approach, it should be considered more frequently in suitable lesions.

Analysis of the treatment planning metrics and their correlation with morphology of intracranial lesions in Gamma Knife stereotactic radiosurgery.

BACKGROUND: Gamma Knife Radiosurgery (GKRS) has established a role in treating various benign brain pathologies. The radiosurgery planning necessitates a proper understanding of radiation dose distribution in relation to the target lesion and surrounding eloquent area. The quality of a radiosurgery plan is determined by various planning parameters. Here, we have reviewed various GKRS planning parameters and analyzed their correlation with the morphology of treated brain lesions. METHOD: A total of 430 treatment plans (71 meningioma, 133 vestibular schwannoma/VS, 150 arteriovenous malformation/AVM, 76 pituitary adenoma/PA treated with GKRS between December 2013 and May 2023) were analyzed for target coverage (TC), conformity index (CI), homogeneity index (HI), and gradient index (GI). RESULT: The values of CIPaddick and CILomax for PA were lower and differed significantly from meningioma, VS, and AVM. The value of HI for PA was higher and differed significantly when compared with meningioma, VS, and AVM. The values of HI for AVM were also significantly higher than VS and meningioma. The mean GI was 3.02, 2.92, 3.03, and 2.88 for meningioma, VS, AVM, and PA, respectively. The value of GI for meningioma and AVM was significantly higher when compared with the values for VS and PA. The mean TC was 0.94 for meningioma, 0.96 for VS, 0.95 for AVM, and 0.90 for PA. The value TC of PA was lower and differed significantly when compared with VS, AVM, and meningioma. Lesions with a volume of ≤1 cc had poor planning metrics as the spillage of radiation may be higher. CONCLUSION: The GKRS planning parameters depend on the size, shape, nature, and location of intracranial lesions. Therefore, each treatment plan needs to be evaluated thoroughly and a long-term follow-up is needed to establish their relation with clinical outcome.