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Future Oncology (London, England) Sep 2018Meningiomas are the most common primary intracranial tumor. Important advances are occurring in meningioma research. These are expected to accelerate, potentially... (Review)
Review
Meningiomas are the most common primary intracranial tumor. Important advances are occurring in meningioma research. These are expected to accelerate, potentially leading to impactful changes on the management of meningiomas in the near and medium term. This review will cover the histo- and molecular pathology of meningiomas, including recent 2016 updates to the WHO classification of CNS tumors. We will discuss clinical and radiographic presentation and therapeutic management. Surgery and radiotherapy, the two longstanding primary therapeutic modalities, will be discussed at length. In addition, data from prior and ongoing investigations of other treatment modalities, including systemic and targeted therapies, will be covered. This review will quickly update the reader on the contemporary management and future directions in meningiomas. [Formula: see text].
Topics: Animals; Biopsy; Combined Modality Therapy; Humans; Meningeal Neoplasms; Meningioma; Multimodal Imaging; Neoplasm Staging; Prognosis; Symptom Assessment; Treatment Outcome
PubMed: 30084265
DOI: 10.2217/fon-2018-0006 -
Journal of Neuro-oncology Sep 2010Although most meningiomas are encapsulated and benign tumors with limited numbers of genetic aberrations, their intracranial location often leads to serious and... (Review)
Review
Although most meningiomas are encapsulated and benign tumors with limited numbers of genetic aberrations, their intracranial location often leads to serious and potentially lethal consequences. They are the most frequently diagnosed primary brain tumor accounting for 33.8% of all primary brain and central nervous system tumors reported in the United States between 2002 and 2006. Inherited susceptibility to meningioma is suggested both by family history and candidate gene studies in DNA repair genes. People with certain mutations in the neurofibromatosis gene (NF2) have a very substantial increased risk for meningioma. High dose ionizing radiation exposure is an established risk factor for meningioma, and lower doses may also increase risk, but which types and doses are controversial or understudied. Because women are twice as likely as men to develop meningiomas and these tumors harbor hormone receptors, an etiologic role for hormones (both endogenous and exogenous) has been hypothesized. The extent to which immunologic factors influence meningioma etiology has been largely unexplored. Growing emphasis on brain tumor research coupled with the advent of new genetic and molecular epidemiologic tools in genetic and molecular epidemiology promise hope for advancing knowledge about the causes of intra-cranial meningioma. In this review, we highlight current knowledge about meningioma epidemiology and etiology and suggest future research directions.
Topics: Humans; Meningeal Neoplasms; Meningioma
PubMed: 20821343
DOI: 10.1007/s11060-010-0386-3 -
Expert Review of Neurotherapeutics Mar 2018With an annual incidence of 5/100,000, meningioma is the most frequent primary tumor of the central nervous system. Risk factors are radiotherapy and hormone intake.... (Review)
Review
With an annual incidence of 5/100,000, meningioma is the most frequent primary tumor of the central nervous system. Risk factors are radiotherapy and hormone intake. Most meningiomas are grade I benign tumors, but up to 15% are atypical and 2% anaplastic according to the WHO 2016 histological criteria. Areas covered: This review details the current standard therapy based on international guidelines and recent literature, and describes new approaches developed to treat refractory cases. First-line treatments are observation and surgery, but adjuvant radiotherapy/radiosurgery is discussed for atypical and indicated for anaplastic meningiomas. The most problematic cases include skull base meningiomas that enclose vasculo-nervous structures and surgery- and radiation-refractory tumors that present with significant morbidity and mortality. The treatment of recurrent tumors is based on radiotherapy and repeated surgery. Systematic therapies are not effective in general but several clinical trials are ongoing. Expert commentary: Molecular characterization of the tumors, based on genetic mutations such as NF2, SMO, TERT, TRAF7, and on the methylation profile are developing, completing the histological classification and giving new insights into prognosis and treatment options.
Topics: Humans; Meningeal Neoplasms; Meningioma; Mutation; Neoplasm Grading; Neoplasm Recurrence, Local; Prognosis; Radiosurgery; Radiotherapy, Adjuvant; Risk Factors; Skull Base Neoplasms
PubMed: 29338455
DOI: 10.1080/14737175.2018.1429920 -
CNS Oncology Jun 2021Meningiomas are the most common primary intracranial tumors. The majority of meningiomas are benign, but they can present different grades of dedifferentiation from... (Review)
Review
Meningiomas are the most common primary intracranial tumors. The majority of meningiomas are benign, but they can present different grades of dedifferentiation from grade I to grade III (anaplastic/malignant) that are associated with different outcomes. Radiological surveillance is a valid option for low-grade asymptomatic meningiomas. In other cases, the treatment is usually surgical, aimed at achieving a complete resection. The use of adjuvant radiotherapy is the gold standard for grade III, is debated for grade II and is not generally indicated for radically resected grade I meningiomas. The use of systemic treatments is not standardized. Here we report a review of the literature on the clinical, radiological and molecular characteristics of meningiomas, available treatment strategies and ongoing clinical trials.
Topics: Brain Neoplasms; Child; Humans; Meningeal Neoplasms; Meningioma; Radiotherapy, Adjuvant
PubMed: 34015955
DOI: 10.2217/cns-2021-0003 -
Neuro-oncology Nov 2021Meningiomas are the most common intracranial tumors. Yet, only few controlled clinical trials have been conducted to guide clinical decision making, resulting in...
Meningiomas are the most common intracranial tumors. Yet, only few controlled clinical trials have been conducted to guide clinical decision making, resulting in variations of management approaches across countries and centers. However, recent advances in molecular genetics and clinical trial results help to refine the diagnostic and therapeutic approach to meningioma. Accordingly, the European Association of Neuro-Oncology (EANO) updated its recommendations for the diagnosis and treatment of meningiomas. A provisional diagnosis of meningioma is typically made by neuroimaging, mostly magnetic resonance imaging. Such provisional diagnoses may be made incidentally. Accordingly, a significant proportion of meningiomas, notably in patients that are asymptomatic or elderly or both, may be managed by a watch-and-scan strategy. A surgical intervention with tissue, commonly with the goal of gross total resection, is required for the definitive diagnosis according to the WHO classification. A role for molecular profiling including gene panel sequencing and genomic methylation profiling is emerging. A gross total surgical resection including the involved dura is often curative. Inoperable or recurrent tumors requiring treatment can be treated with radiosurgery, if the size or the vicinity of critical structures allows that, or with fractionated radiotherapy (RT). Treatment concepts combining surgery and radiosurgery or fractionated RT are increasingly used, although there remain controversies regard timing, type, and dosing of the various RT approaches. Radionuclide therapy targeting somatostatin receptors is an experimental approach, as are all approaches of systemic pharmacotherapy. The best albeit modest results with pharmacotherapy have been obtained with bevacizumab or multikinase inhibitors targeting vascular endothelial growth factor receptor, but no standard of care systemic treatment has been yet defined.
Topics: Aged; Humans; Magnetic Resonance Imaging; Meningeal Neoplasms; Meningioma; Radiosurgery; Vascular Endothelial Growth Factor A
PubMed: 34181733
DOI: 10.1093/neuonc/noab150 -
Journal of Clinical Oncology : Official... Dec 2021Meningiomas are the most frequent primary intracranial tumors. Patient outcome varies widely from benign to highly aggressive, ultimately fatal courses. Reliable...
PURPOSE
Meningiomas are the most frequent primary intracranial tumors. Patient outcome varies widely from benign to highly aggressive, ultimately fatal courses. Reliable identification of risk of progression for individual patients is of pivotal importance. However, only biomarkers for highly aggressive tumors are established ( and ), whereas no molecularly based stratification exists for the broad spectrum of patients with low- and intermediate-risk meningioma.
METHODS
DNA methylation data and copy-number information were generated for 3,031 meningiomas (2,868 patients), and mutation data for 858 samples. DNA methylation subgroups, copy-number variations (CNVs), mutations, and WHO grading were analyzed. Prediction power for outcome was assessed in a retrospective cohort of 514 patients, validated on a retrospective cohort of 184, and on a prospective cohort of 287 multicenter cases.
RESULTS
Both CNV- and methylation family-based subgrouping independently resulted in increased prediction accuracy of risk of recurrence compared with the WHO classification (c-indexes WHO 2016, CNV, and methylation family 0.699, 0.706, and 0.721, respectively). Merging all risk stratification approaches into an integrated molecular-morphologic score resulted in further substantial increase in accuracy (c-index 0.744). This integrated score consistently provided superior accuracy in all three cohorts, significantly outperforming WHO grading (c-index difference = .005). Besides the overall stratification advantage, the integrated score separates more precisely for risk of progression at the diagnostically challenging interface of WHO grade 1 and grade 2 tumors (hazard ratio 4.34 [2.48-7.57] and 3.34 [1.28-8.72] retrospective and prospective validation cohorts, respectively).
CONCLUSION
Merging these layers of histologic and molecular data into an integrated, three-tiered score significantly improves the precision in meningioma stratification. Implementation into diagnostic routine informs clinical decision making for patients with meningioma on the basis of robust outcome prediction.
Topics: Humans; Meningioma; Prospective Studies; Retrospective Studies
PubMed: 34618539
DOI: 10.1200/JCO.21.00784 -
Neuroendocrinology 2020Parasellar spaces remain particularly singular, comprising the most important neurovascular structures such as the internal carotid artery and optic, oculomotor, and... (Review)
Review
Parasellar spaces remain particularly singular, comprising the most important neurovascular structures such as the internal carotid artery and optic, oculomotor, and trigeminal nerves. Meningiomas are one of the most frequent tumors arising from parasellar spaces. In this location, meningiomas remain mostly benign tumors with WHO grade I and a meningothelial subtype. Progestin intake should be investigated and leads mostly to conservative strategies. In the case of benign nonsymptomatic tumors, observation should be proposed. Tumor growth will lead to the proposition of surgery or radiosurgery. In the case of an uncertain diagnosis and an aggressive pattern, a precise diagnosis is required. For cavernous sinus and Meckel's cave lesions, complete removal is rarely considered, leading to the proposition of an endoscopic endonasal or transcranial biopsy. Optic nerve decompression could also be proposed via these approaches. A case-by-case discussion about the best approach is recommended. A transcranial approach remains necessary for tumor removal in most cases. Vascular injury could lead to severe complications. Cerebrospinal fluid leakage, meningitis, venous sacrifice, visual impairment, and cranial nerve palsies are more frequent complications. Pituitary dysfunctions are rare in preoperative assessment and in postoperative follow-up but should be assessed in the case of meningiomas located close to the pituitary axis. Long-term follow-up is required given the frequent incomplete tumor removal and the risk of delayed recurrence. Radiosurgery is relevant for small and well-limited meningiomas or intra-cavernous sinus postoperative residue, whereas radiation therapy and proton beam therapy are indicated for large, extended, nonoperable meningiomas. The place of the peptide receptor radionuclide therapyneeds to be defined. Targeted therapy should be considered in rare, recurrent, and aggressive parasellar meningiomas.
Topics: Cavernous Sinus; Cranial Nerve Neoplasms; Humans; Meningeal Neoplasms; Meningioma; Skull Base Neoplasms
PubMed: 32492684
DOI: 10.1159/000509090 -
Ideggyogyaszati Szemle Jul 2016Meningiomas are one of the most frequent primary intracranial tumours, representing one-third of all intracranialneoplasms. The vast majority of meningiomas are... (Review)
Review
Meningiomas are one of the most frequent primary intracranial tumours, representing one-third of all intracranialneoplasms. The vast majority of meningiomas are histologically benign, but recurrence and progression is quite frequent. They occur usually between the 6th and 7th decade, the female/male ratio is 3:2. Although rare in pregnancy, when occurring, they can cause serious, life-threatening complications due to rapid growth and unfavourable localisation. There are two dominant hypothesis explaining rapid growth in pregnancy: the role of hormonal effects and hemodynamic changes. Several studies tested these theories but none provided unequivocal answer probably because the pathomechanism is complex and multifactorial. We provide an overview of the pathomechanism of meningiomas in pregnancy with emphasis on data obtained by advanced neuropathological, molecular biological, bioinformatic, imaging and epidemiological methods. A better understanding of the processes leading to meningioma development and growth in pregnancy will help us to design personalized therapy and reduce morbidity and mortality.
Topics: Adult; Female; Humans; Meningeal Neoplasms; Meningioma; Pregnancy; Pregnancy Complications, Neoplastic
PubMed: 29465885
DOI: 10.18071/isz.69.0220 -
BMJ Case Reports Feb 2015Cystic meningioma is an uncommon form of meningioma and the radiological appearance and location of the cystic/solid components of the mass may create a diagnostic...
Cystic meningioma is an uncommon form of meningioma and the radiological appearance and location of the cystic/solid components of the mass may create a diagnostic dilemma. We report on the case of an 80-year-old left-handed man who presented with recurrent focal motor seizures and secondary generalisation. A large left-sided parieto-occipital cystic mass was detected on contrast CT of the brain. The appearance suggested a malignant tumour. However, brain MRI with gadolinium delineated the solid and cystic component precisely. A provisional diagnosis of cystic meningioma was made and this was confirmed histologically after resecting the tumour surgically. It was a benign WHO Grade I fibroblastic meningioma. The preoperative diagnosis of cystic meningioma is not that straightforward. Brain MRI with gadolinium has a better diagnostic yield than CT scanning. Histopathological examination of the tumour cells should always be performed to confirm the category and subtype of the tumour.
Topics: Aged, 80 and over; Brain Neoplasms; Cysts; Diagnosis, Differential; Humans; Magnetic Resonance Imaging; Male; Meningioma; Occipital Lobe; Parietal Lobe; Tomography, X-Ray Computed
PubMed: 25670784
DOI: 10.1136/bcr-2014-207690 -
Radiation Oncology (London, England) Dec 2017The rapid rise of particle therapy across the world necessitates evidence to justify its ever-increasing utilization. This narrative review summarizes the current status... (Review)
Review
The rapid rise of particle therapy across the world necessitates evidence to justify its ever-increasing utilization. This narrative review summarizes the current status of these technologies on treatment of both meningiomas and gliomas, the most common benign and malignant primary brain tumors, respectively. Proton beam therapy (PBT) for meningiomas displays high rates of long-term local control, low rates of symptomatic deterioration, along with the potential for safe dose-escalation in select (but not necessarily routine) cases. PBT is also associated with low adverse events and maintenance of functional outcomes, which have implications for quality of life and cost-effectiveness measures going forward. Data on carbon ion radiation therapy (CIRT) are limited; existing series describe virtually no high-grade toxicities and high local control. Regarding the few available data on low-grade gliomas, PBT provides opportunities to dose-escalate while affording no increase of severe toxicities, along with maintaining appropriate quality of life. Although dose-escalation for low-grade disease has been less frequently performed than for glioblastoma, PBT and CIRT continue to be utilized for the latter, and also have potential for safer re-irradiation of high-grade gliomas. For both neoplasms, the impact of superior dosimetric profiles with endpoints such as neurocognitive decline and neurologic funcionality, are also discussed to the extent of requiring more data to support the utility of particle therapy. Caveats to these data are also described, such as the largely retrospective nature of the available studies, patient selection, and heterogeneity in patient population as well as treatment (including mixed photon/particle treatment). Nevertheless, multiple prospective trials (which may partially attenuate those concerns) are also discussed. In light of the low quantity and quality of available data, major questions remain regarding economic concerns as well.
Topics: Glioma; Heavy Ion Radiotherapy; Humans; Meningioma; Proton Therapy; Treatment Outcome
PubMed: 29195506
DOI: 10.1186/s13014-017-0924-7