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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 -
Critical Reviews in Oncology/hematology Aug 2008Meningiomas are mostly benign tumours originating from the arachnoid cap cells, represent 13-26% of all intracranial tumours. They are more common in older age and in... (Review)
Review
Meningiomas are mostly benign tumours originating from the arachnoid cap cells, represent 13-26% of all intracranial tumours. They are more common in older age and in females. Deletion in NF2 gene and exposure to ionizing radiation are established risk factors, while the role of sex hormones is yet not clarified. Five-year survival for typical meningiomas exceeds 80%, but is poorer (5-year survival <60%) in malignant and atypical meningiomas. Papillary and haemangiopericytic morphology, large tumour size, high mitotic index, absence of progesterone receptors, deletions and loss of heterozygosity are poor prognostic factors. Complete surgical excision is the standard treatment. Radiotherapy is currently used in the clinical practice in atypical, malignant or recurrent meningioma at a total dose of 45-60Gy. However, the role of adjuvant irradiation is still controversial and has to be compared in a randomised prospective setting with a policy of watchful waiting. Radiosurgery has gained more and more importance in the management of meningiomas, especially in meningiomas that cannot be completely resected as for many skull base meningiomas. Medical therapy for patients with recurrent, progressive and symptomatic disease after repeated surgery, radiosurgery and radiotherapy is investigational. Hormonal therapy with progesterone antagonists has shown modest results, while chemotherapy with hydroxyurea appears moderately active.
Topics: Combined Modality Therapy; Humans; Meningeal Neoplasms; Meningioma; Neoplasm Staging; Prognosis; Risk Factors
PubMed: 18342535
DOI: 10.1016/j.critrevonc.2008.01.010 -
Journal of Integrative Neuroscience Jun 2021Meningiomas are amongst the most commonly encountered intracranial tumors. The majority of these tumors arise intracranially, and the remaining incidents occur along the... (Review)
Review
Meningiomas are amongst the most commonly encountered intracranial tumors. The majority of these tumors arise intracranially, and the remaining incidents occur along the spinal cord. Meningiomas tend to grow gradually, with many tumors arising in inaccessible locations. Such sporadic behavior poses a therapeutic challenge to clinicians, causing incomplete tumor resections that often lead to recurrence. Therefore, ongoing research seeks to find alternative systematic treatments for meningiomas, with gene-based therapeutics of high interest. Subsequently, genetic studies characterized frequent somatic mutations in , , , , , and . These genes are communally exhibited in 80% of sporadic meningiomas. In addition, other genes such as the family, the family, , and , have been identified as key players in spinal meningiomas. In this perspective, we aim to investigate current genetic-based studies, with the ongoing research mainly focused on the above , , , , , and genes and their involved pathways. In addition, this perspective can serve as a potential cornerstone for future genetic analyses of meningioma cases.
Topics: Humans; Meningeal Neoplasms; Meningioma
PubMed: 34258948
DOI: 10.31083/j.jin2002049 -
Journal of Neuro-oncology Sep 2010Meningiomas, although mostly benign, may sometimes present aggressive features and raise issues concerning alternative treatment options besides surgery. In order to... (Review)
Review
Meningiomas, although mostly benign, may sometimes present aggressive features and raise issues concerning alternative treatment options besides surgery. In order to gain new insights in meningioma biology and develop alternative treatments, several meningioma mouse models have been engineered during the past two decades. As rodents very rarely develop spontaneous meningiomas, animal models have been first developed by implanting human meningioma cells derived from a primary tumor and meningioma cell lines subcutaneously into athymic mice. Induction of de novo meningiomas in rodents with mutagens, such as nitrosourea, has also been reported. Advances in our understanding of molecular genetics of meningioma have pinpointed the central role of NF2 tumor suppressor gene in the pathogenesis of those tumors. These discoveries have led to the creation of a genetically engineered model utilizing conditional mutagenesis to specifically inactivate the mouse Nf2 gene in arachnoidal cells, resulting in the formation of intracranial meningothelial hyperplasia and meningiomas and thus reproducing the main mechanism of human meningeal tumorigenesis. This powerful new technology significantly improves on prior models and may open avenues of investigation never before possible in meningioma research. We present here a review of current meningioma mouse models used in translational therapeutics with associated imaging and pre-clinical studies.
Topics: Animals; Disease Models, Animal; Humans; Meningeal Neoplasms; Meningioma; Mice
PubMed: 20734219
DOI: 10.1007/s11060-010-0331-5 -
Neurosurgery Clinics of North America Apr 2016Primary intraosseous meningiomas are a subtype of primary extradural meningiomas. They represent approximately two-thirds of extradural meningiomas and fewer than 2% of... (Review)
Review
Primary intraosseous meningiomas are a subtype of primary extradural meningiomas. They represent approximately two-thirds of extradural meningiomas and fewer than 2% of meningiomas overall. These tumors originate within the bones of the skull and can have a clinical presentation and radiographic differential diagnosis different from those for intradural meningiomas. Primary intraosseous meningiomas are classified based on location and histopathologic characteristics. Treatment is primarily surgical resection with wide margins if possible. Sparse literature exists regarding the use of adjuvant therapies. The literature regarding primary intraosseous meningiomas consists primarily of clinical case reports and case series. This literature is reviewed and summarized in this article.
Topics: Humans; Meningeal Neoplasms; Meningioma; Skull
PubMed: 27012383
DOI: 10.1016/j.nec.2015.11.011 -
Current Opinion in Oncology Nov 2020To discuss recent advances in the meningioma biology and their clinical implications. (Review)
Review
PURPOSE OF REVIEW
To discuss recent advances in the meningioma biology and their clinical implications.
RECENT FINDINGS
Meningioma is the most common primary intracranial tumor. Mostly benign, 20% of cases display an aggressive behavior despite best standard of care. The genetic landscape of meningiomas is divided according to NF2 mutational status. Although about 60% of meningiomas display NF2 mutations, the other share is more heterogenous. Mutations in TRAF7, SMO, v-akt murine thymoma viral oncogene homolog 1 (AKT1), PI3KCA and KLF4 are seen mostly in WHO grade 1 meningiomas. In higher grade meningiomas, mutations of the TERT promoter and deletions of CDKN2A/B emerge and have prognostic value. Moreover, mutations in DMD, BAP1 and PBRM1 have recently been discovered and are being further explored. DNA methylation subgroups offer valuable insight into meningioma prognosis and its implementation in clinical setting is under evaluation. Moreover, the study of distinct meningioma populations such as radiation-induced meningioma and progestin-associated meningioma may provide further insight into meningioma oncogenesis and potential therapeutic targets.
SUMMARY
The mutational landscape of meningioma has expanded following the use of the new genetic sequencing approaches. Novel mutations have been characterized and reveal their prognostic and therapeutic applications. This improved understanding of meningioma biology has promising implications for novel treatment strategies.
Topics: Epigenesis, Genetic; Humans; Kruppel-Like Factor 4; Meningeal Neoplasms; Meningioma; Prognosis
PubMed: 32890025
DOI: 10.1097/CCO.0000000000000687 -
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 -
Nature Reviews. Neurology Feb 2018Meningiomas currently are among the most frequent intracranial tumours. Although the majority of meningiomas can be cured by surgical resection, ∼20% of patients have... (Review)
Review
Meningiomas currently are among the most frequent intracranial tumours. Although the majority of meningiomas can be cured by surgical resection, ∼20% of patients have an aggressive clinical course with tumour recurrence or progressive disease, resulting in substantial morbidity and increased mortality of affected patients. During the past 3 years, exciting new data have been published that provide insights into the molecular background of meningiomas and link sites of tumour development with characteristic histopathological and molecular features, opening a new road to novel and promising treatment options for aggressive meningiomas. A growing number of the newly discovered recurrent mutations have been linked to a particular clinicopathological phenotype. Moreover, the updated WHO classification of brain tumours published in 2016 has incorporated some of these molecular findings, setting the stage for the improvement of future therapeutic efforts through the integration of essential molecular findings. Finally, an additional potential classification of meningiomas based on methylation profiling has been launched, which provides clues in the assessment of individual risk of meningioma recurrence. All of these developments are creating new prospects for effective molecularly driven diagnosis and therapy of meningiomas.
Topics: Animals; Humans; Meningeal Neoplasms; Meningioma
PubMed: 29302064
DOI: 10.1038/nrneurol.2017.168 -
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 -
American Journal of Otolaryngology 2022Meningiomas are tumors that arise from arachnoid cells attached to both the pia mater and the inner portion of the arachnoid. They are common intracranial tumors,... (Review)
Review
Meningiomas are tumors that arise from arachnoid cells attached to both the pia mater and the inner portion of the arachnoid. They are common intracranial tumors, representing 12-25% of intracranial neoplasms. Intracranial meningiomas can spread extracranially to involve surrounding structures, including the ear and temporal bone. Ectopic meningiomas, described as primary meningiomas with no intracranial involvement, are rare. We describe a case of a primary external auditory canal meningioma with no evidence of intracranial involvement. We present pre-operative imaging findings proving no intracranial involvement prior to surgical intervention. A literature review of this uncommon clinical entity is presented and a discussion regarding its prognosis and treatment is reviewed.
Topics: Ear Canal; Ear Neoplasms; Humans; Meningioma; Prognosis; Rare Diseases
PubMed: 34536924
DOI: 10.1016/j.amjoto.2021.103215