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.

[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.

Primary Pulmonary Meningioma With Associated Multiple Micronodules: A Case Report With Comprehensive Diagnostic Overview.

BACKGROUND: Primary pulmonary meningioma (PPM) is an exceedingly rare neoplasm originating in the meninges within the lung. Despite sharing similarities with its central nervous system (CNS) counterparts, PPM presents unique diagnostic challenges and therapeutic considerations owing to its infrequent occurrence. CASE: This case report describes a 73-year-old male who underwent chest computed tomography (CT), which revealed a mass in the posterior basal segment of the right lower lobe, suggestive of a low-grade malignant tumor approximately 30-40 mm in size. Single-port video-assisted thoracoscopic surgery (VATS) was performed to resect the mass via localized lesion excision (lung wedge resection). Intraoperative frozen section pathology indicated a low-grade malignant epithelial tumor, leading to a decision for maximal lung function preservation, considering the patient's advanced age. The surgical team opted for a localized excision to ensure negative margins. Histopathological analysis confirmed the diagnosis of epithelioid PPM, a rare subtype even among PPM cases (World Health Organization [WHO] Grade I). The patient was discharged 9 days after surgery without complications and resumed normal daily activities 1 month postoperatively. The rarity of PPM precludes a standardized treatment protocol, with surgical resection as the primary approach. However, the efficacy of adjunctive therapies remains uncertain due to limited evidence. CONCLUSION: This case report contributes to a better understanding of PPM and emphasizes the importance of a comprehensive diagnostic evaluation and individualized treatment planning for this rare entity.

In situ brain tumor detection using a Raman spectroscopy system-results of a multicenter study.

Safe and effective brain tumor surgery aims to remove tumor tissue, not non-tumoral brain. This is a challenge since tumor cells are often not visually distinguishable from peritumoral brain during surgery. To address this, we conducted a multicenter study testing whether the Sentry System could distinguish the three most common types of brain tumors from brain tissue in a label-free manner. The Sentry System is a new real time, in situ brain tumor detection device that merges Raman spectroscopy with machine learning tissue classifiers. Nine hundred and seventy-six in situ spectroscopy measurements and colocalized tissue specimens were acquired from 67 patients undergoing surgery for glioblastoma, brain metastases, or meningioma to assess tumor classification. The device achieved diagnostic accuracies of 91% for glioblastoma, 97% for brain metastases, and 96% for meningiomas. These data show that the Sentry System discriminated tumor containing tissue from non-tumoral brain in real time and prior to resection.

Biomarkers for prognosis of meningioma patients: A systematic review and meta-analysis.

Meningioma is the most common primary brain tumor and many studies have evaluated numerous biomarkers for their prognostic value, often with inconsistent results. Currently, no reliable biomarkers are available to predict the survival, recurrence, and progression of meningioma patients in clinical practice. This study aims to evaluate the prognostic value of immunohistochemistry-based (IHC) biomarkers of meningioma patients. A systematic literature search was conducted up to November 2023 on PubMed, CENTRAL, CINAHL Plus, and Scopus databases. Two authors independently reviewed the identified relevant studies, extracted data, and assessed the risk of bias of the studies included. Meta-analyses were performed with the hazard ratio (HR) and 95% confidence interval (CI) of overall survival (OS), recurrence-free survival (RFS), and progression-free survival (PFS). The risk of bias in the included studies was evaluated using the Quality in Prognosis Studies (QUIPS) tool. A total of 100 studies with 16,745 patients were included in this review. As the promising markers to predict OS of meningioma patients, Ki-67/MIB-1 (HR = 1.03, 95%CI 1.02 to 1.05) was identified to associate with poor prognosis of the patients. Overexpression of cyclin A (HR = 4.91, 95%CI 1.38 to 17.44), topoisomerase II α (TOP2A) (HR = 4.90, 95%CI 2.96 to 8.12), p53 (HR = 2.40, 95%CI 1.73 to 3.34), vascular endothelial growth factor (VEGF) (HR = 1.61, 95%CI 1.36 to 1.90), and Ki-67 (HR = 1.33, 95%CI 1.21 to 1.46), were identified also as unfavorable prognostic biomarkers for poor RFS of meningioma patients. Conversely, positive progesterone receptor (PR) and p21 staining were associated with longer RFS and are considered biomarkers of favorable prognosis of meningioma patients (HR = 0.60, 95% CI 0.41 to 0.88 and HR = 1.89, 95%CI 1.11 to 3.20). Additionally, high expression of Ki-67 was identified as a prognosis biomarker for poor PFS of meningioma patients (HR = 1.02, 95%CI 1.00 to 1.04). Although only in single studies, KPNA2, CDK6, Cox-2, MCM7 and PCNA are proposed as additional markers with high expression that are related with poor prognosis of meningioma patients. In conclusion, the results of the meta-analysis demonstrated that PR, cyclin A, TOP2A, p21, p53, VEGF and Ki-67 are either positively or negatively associated with survival of meningioma patients and might be useful biomarkers to assess the prognosis.

Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue of the dura mimicking meningioma: a case report and literature review.

MALT lymphoma of the dura is a very rare type of low-grade B-cell lymphoma. Little more than 100 cases have been reported in the literature to date. We report a 43-year-old woman who was referred to hospital because of a series of three tonic-clonic seizures on the day of admission. Neurological examination revealed confusion and aphasia. Magnetic resonance imaging (MRI) showed a contrast-enhanced, broad-based lesion along the dura in the left parieto-occipital area. The suspicion of an en plaque meningioma was raised. The tumour invaded the brain parenchyma with visible extension into the brain sulci. There was a marked brain oedema surrounding the lesion and causing the midline shift 8 mm to the right. After stabilization of neurological condition (intravenous diuretics and steroids), the operation was performed. The diagnosis of dural MALT lymphoma was established. During the pathological examination, it was especially problematic to distinguish MALT lymphoma from follicular lymphoma, but the final diagnosis was MALT lymphoma. Surgical partial removal with additional R-CVP immunochemotherapy (rituximab, cyclophosphamide, vincristine and prednisone) resulted in complete remission. The follow-up period is 1 year. Our presented case of a MALT lymphoma highlights the fact that surgical partial removal with additional immunochemotherapy is an available option in these rare intracranial tumours.

Innovation in Non-Invasive Diagnosis and Disease Monitoring for Meningiomas.

Meningiomas are tumors of the central nervous system that vary in their presentation, ranging from benign and slow-growing to highly aggressive. The standard method for diagnosing and classifying meningiomas involves invasive surgery and can fail to provide accurate prognostic information. Liquid biopsy methods, which exploit circulating tumor biomarkers such as DNA, extracellular vesicles, micro-RNA, proteins, and more, offer a non-invasive and dynamic approach for tumor classification, prognostication, and evaluating treatment response. Currently, a clinically approved liquid biopsy test for meningiomas does not exist. This review provides a discussion of current research and the challenges of implementing liquid biopsy techniques for advancing meningioma patient care.

FNA of Meningioma with Rhabdoid Features Presenting as a Lateral Neck Mass.

Primary meningioma at extracranial head and neck sites is uncommon. Since fine needle aspiration (FNA) is often the first line diagnostic modality for the evaluation of masses in the head and neck, extracranial meningiomas can create a significant diagnostic pitfall for FNA. We report a case of meningioma with rhabdoid features and BAP1 loss in a 26-year-old woman, presenting as a large neck mass along the carotid sheath. FNA biopsy of the mass demonstrated a highly cellular specimen with clusters of uniform, epithelioid cells with round to ovoid nuclei and moderate nuclear to cytoplasmic ratio. An extensive immunohistochemical panel performed on cell block sections showed that the tumor cells were weakly EMA positive, progesterone receptor was focally positive, and SSTR2A was diffuse and strongly positive. BAP1 immunohistochemistry showed a diffuse loss of expression in the tumor cells. After the cytologic diagnosis of meningioma, a tissue biopsy was performed, and the diagnosis of meningioma with rhabdoid features and BAP1 loss was confirmed. We also perform a literature review of meningioma cases presenting as a neck mass and evaluated by FNA. Our case highlights the significant diagnostic challenges that can be caused by extracranial meningiomas on FNA and the importance of ancillary studies to avoid diagnostic pitfalls.

Case report of a primary ectopic extradural and extraspinal meningioma of the brachial plexus.

Primary ectopic extradural and extraspinal meningiomas are rare. We present a unique case of this type of meningioma in the brachial plexus. A 25-year-old man consulted us because of neuropathic supraclavicular pain and the appearance of a supraclavicular mass whose volume had increased. Clinical examination found paresis of the deltoid, biceps brachii and brachialis muscles rated as M4 (MRC) and a strong Tinel sign at the supraclavicular fossa, over the palpable mass. There was no sign pointing towards central nervous system involvement or altered general condition. MRI revealed a mass measuring 53 × 24 mm invading the C5-C6 plexus roots and the primary upper trunk, but not the bone or spinal area. This lesion was hyperintense on DWI/ADC, hyperintense on T2 with hypointense spots, and hypointense on T1 with intense heterogeneous gadolinium enhancement. Excisional biopsy was done 6 months after symptoms started. The tumor had developed at the C5 root, which was fibrous and at the C6 root, which was grossly normal. Anatomical pathology confirmed the WHO grade 1 meningioma, meningothelial and psammomatous histological subtypes. At 6 months, a follow-up MRI found no postoperative tumor remnants or recurrence. During the postoperative course, persistent paralysis of the deltoid muscle at 5 months justified a nerve transfer. This is a rare case of ectopic extraspinal and extradural meningioma of the brachial plexus. The diagnosis of an ectopic meningioma must be considered when a patient presents with a brachial plexus tumor causing neurological deficits. The extradural nature is not sufficient to rule out this diagnosis.

FT-Raman spectra in combination with machine learning and multivariate analyses as a diagnostic tool in brain tumors.

Brain tumors are one of the most dangerous, because the position of these are in the organ that governs all life processes. Moreover, a lot of brain tumor types were observed, but only one main diagnostic method was used - histopathology, for which preparation of sample was long. Consequently, a new, quicker diagnostic method is needed. In this paper, FT-Raman spectra of brain tissues were analyzed by Principal Component Analysis (PCA), Hierarchical Cluster Analysis (HCA), four different machine learning (ML) algorithms to show possibility of differentiating between glioblastoma G4 and meningiomas, as well as two different types of meningiomas (atypical and angiomatous). Obtained results showed that in meningiomas additional peak around 1503 cm-1 and higher level of amides was noticed in comparison with glioblastoma G4. In the case of meningiomas differentiation, in angiomatous meningiomas tissues lower level of lipids and polysaccharides were visible than in atypical meningiomas. Moreover, PCA analyses showed higher distinction between glioblastoma G4 and meningiomas in the FT-Raman range between 800 cm-1 and 1800 cm-1 and between two types of meningiomas in the range between 2700 cm-1 and 3000 cm-1. Decision trees showed, that the most important peaks to differentiate glioblastoma and meningiomas were at 1151 cm-1 and 2836 cm-1 while for angiomatous and atypical meningiomas - 1514 cm-1 and 2875 cm-1. Furthermore, the accuracy of obtained results for glioblastoma G4 and meningiomas was 88 %, while for meningiomas - 92 %. Consequently, obtained data showed possibility of using FT-Raman spectroscopy in diagnosis of different types of brain tumors.

Lumbar clear cell meningioma mimicking schwannoma 7 years after resection of the same type of intracranial tumor: a case report.

BACKGROUND: Meningioma is the second most common intradural extramedullary tumor, following schwannoma. Meningioma is primarily categorized as benign World Health Organization grade 1, but clear cell meningioma is grade 2 of the intermediate malignant category. Clear cell meningiomas are rare, accounting for less than 1% of all meningioma tumors. There is no previous report of multiple intraspinal clear cell meningiomas without dural attachment. CASE PRESENTATION: A 27-year-old Asian male patient presented with lower right extremity pain, and had undergone tumor resection for intracranial clear cell meningioma 7 years previously, with re-resection and radiotherapy for local tumor recurrence at our hospital's department of neurosurgery being carried out 4 years previously. No recurrence was observed since then. Preoperative lumbar magnetic resonance imaging showed two tumors at the L1 and L4 levels, both mimicking schwannoma with well-defined margins, no dural tail sign and homogeneous internal contrast. Intraoperative findings on tumor resection showed two tumors contiguous with the right L2 and L5 roots, which were not attached to the dura mater, similar to a schwannoma. After gross total resection, the postoperative pathology revealed no nuclear SMARCE1 antibody staining. The patient was diagnosed with clear cell meningioma. The patient's postoperative course went well, with no symptoms of nerve dropout and no recurrence 2 years after surgery. In this case, both lumbar lesions were well demarcated and spherical in shape, occurring with single roots. Tumor characteristics suggested a primary rather than a metastatic lesion. Clear cell meningioma is characterized by a SMARCE1 mutation and is different from other types of meningiomas. CONCLUSION: To the best of our knowledge, this is the first report of multiple intraspinal clear cell meningiomas without dural attachment at the lumbar spine after resection of intracranial clear cell meningioma. We speculate that the two tumors were de novo lesions on the basis of the features of the tumors, although they were detected 7 years after the resection of intracranial clear cell meningioma.

Tuberculum meningioma with recovery of glaucoma-like visual field defects after chiasmal decompression: a case report.

BACKGROUND: To report a case of tuberculum meningioma with recovery of glaucoma-like visual field defects after chiasmal decompression. CASE PRESENTATION: A 39-year-old woman presenting with headache was found to have bilateral arcuate retinal nerve fiber layer (RNFL) thinning on optical coherence tomography (OCT) with a corresponding arcuate scotomas consistent with glaucomatous change. However a suprasellar tumor compressing the anterior chiasm from below was found on magnetic resonance imaging of the brain. After resection of the mass, which was diagnosed as meningothelial meningioma by the pathological examination, the glaucoma-like visual field defects resolved despite the RNFL thinning on the OCT showing no improvement. CONCLUSIONS: Chiasmal compression may mimic glaucoma and produce arcuate scotoma rather than temporal visual field loss. There is a possibility that the development of chiasmal compression somehow converted preperimetric glaucoma into a more advanced form accompanied by visual field defects and that the glaucoma reverted to the preperimetric state after chiasmal decompression.

Metastasis infiltrating tumor to meningioma: a case report.

BACKGROUND: There have been many reports of tumor-to-tumor metastasis, in which cancer metastasizes directly into meningiomas. However, metastasis infiltrating tumors in which cancer metastasizes around meningiomas are rare. Therefore, we report a case of metastasis originating from lung cancer that infiltrated meningioma. CASE PRESENTATION: A 79-year-old Japanese woman underwent head magnetic resonance imaging for brain metastasis screening before lung cancer surgery. At that time, asymptomatic meningioma of the left frontal region was accidentally found. Magnetic resonance imaging 6 months later revealed a lesion suspected to be a metastatic brain tumor close to the meningioma. Brain tumor resection was performed, and histopathological diagnosis was meningioma and metastatic brain tumor. Metastatic cancer had invaded the meningioma at the boundary between the brain tumor and metastasis. CONCLUSIONS: A sudden change in imaging findings on routine examination of meningiomas in patients with lung carcinoma may indicate a metastatic brain tumor. The form of cancer metastasis to meningioma is not limited to tumor-to-tumor metastasis, but also includes metastasis infiltrating tumors near the meningioma.