-
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 -
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 -
Handbook of Clinical Neurology 2020Radiation-induced meningiomas (RIMs) became more common as the use of ionizing radiation was adopted in the treatment of medical conditions, both benign and malignant....
Radiation-induced meningiomas (RIMs) became more common as the use of ionizing radiation was adopted in the treatment of medical conditions, both benign and malignant. Currently, RIMs represent the most common radiation-induced tumors. They are heterogeneous in terms of patient characteristics, radiographic appearance, genetics, pathology, symptoms, and management strategies. They tend to occur in a younger population and are generally more aggressive in nature than their spontaneous counterparts. Their characteristics also vary based on the dose of radiation received, which is most commonly separated into low dose (<10Gy) and high dose (>10Gy). The importance of the dosing classification is that it can provide insight into the nature and biologic behavior of the tumor. Given their heterogeneity, RIMs pose significant challenges in management. While surgical resection remains the preferred treatment when feasible, recent data supports stereotactic radiosurgery (SRS) as a comparable alternative. Although there is more knowledge about the molecular pathways leading to RIMs, targeted drug therapy is still limited and is the focus of current research.
Topics: Adult; Female; Humans; Male; Meningeal Neoplasms; Meningioma; Middle Aged; Neoplasms, Radiation-Induced; Radiosurgery; Treatment Outcome
PubMed: 32553295
DOI: 10.1016/B978-0-12-804280-9.00018-4 -
World Neurosurgery Dec 2022Melanocytes are neural crest derivatives. Intracranial meningeal melanocytomas (MM) are rare tumors and the available literature is sparse for these neoplasms. This... (Review)
Review
OBJECTIVE
Melanocytes are neural crest derivatives. Intracranial meningeal melanocytomas (MM) are rare tumors and the available literature is sparse for these neoplasms. This review aims to provide a detailed and comprehensive literature review of these tumors.
METHODS
By searching online databases, a literature review was performed to include and analyze all cases of cranial MM reported to date. PRISMA guidelines were followed for the review process.
RESULTS
A total of 109 cases were analyzed. The male/female ratio was 1.2:1. The mean age was 40.3 years. The mean duration of symptoms was 23.9 months (range, 2 days-15 years). Cerebellopontine (CP) angle, suprasellar, and Meckel cave were the most common locations. Sixty-four patients (58%) underwent gross total resection (GTR). Twenty-eight patients (26%) received some form of adjuvant radiation. There were 29 recurrences (26%) and mean time to recurrence was 50.2 months. The recurrence rates (RRs) for GTR and subtotal resection were 20% and 42%, respectively. The extent of resection (subtotal resection) and tumor locations (CP angle and Meckel cave) were significantly associated with higher RR. Six patients (6.5%) had higher-grade transformations.
CONCLUSIONS
Complete surgical resection is the ideal treatment and adjuvant radiation is to be considered for residual/recurrent tumors. Adjuvant radiotherapy may also be prescribed despite GTR, in locations with higher RR such as CP angle and Meckel cave. Because of higher-grade transformations and delayed recurrences, long-term follow-up is required.
Topics: Adult; Humans; Female; Male; Neoplasm Recurrence, Local; Meningeal Neoplasms; Nevus, Pigmented; Melanocytes; Skin Neoplasms
PubMed: 35977682
DOI: 10.1016/j.wneu.2022.08.051 -
Neuro-oncology Jan 2019Meningiomas are the most common primary intracranial neoplasm. The current World Health Organization (WHO) classification categorizes meningiomas based on... (Review)
Review
Meningiomas are the most common primary intracranial neoplasm. The current World Health Organization (WHO) classification categorizes meningiomas based on histopathological features, but emerging molecular data demonstrate the importance of genomic and epigenomic factors in the clinical behavior of these tumors. Treatment options for symptomatic meningiomas are limited to surgical resection where possible and adjuvant radiation therapy for tumors with concerning histopathological features or recurrent disease. At present, alternative adjuvant treatment options are not available in part due to limited historical biological analysis and clinical trial investigation on meningiomas. With advances in molecular and genomic techniques in the last decade, we have witnessed a surge of interest in understanding the genomic and epigenomic landscape of meningiomas. The field is now at the stage to adopt this molecular knowledge to refine meningioma classification and introduce molecular algorithms that can guide prediction and therapeutics for this tumor type. Animal models that recapitulate meningiomas faithfully are in critical need to test new therapeutics to facilitate rapid-cycle translation to clinical trials. Here we review the most up-to-date knowledge of molecular alterations that provide insight into meningioma behavior and are ready for application to clinical trial investigation, and highlight the landscape of available preclinical models in meningiomas.
Topics: Combined Modality Therapy; Genomics; Humans; Meningeal Neoplasms; Meningioma; Molecular Targeted Therapy; Prognosis; Translational Research, Biomedical
PubMed: 30649490
DOI: 10.1093/neuonc/noy178 -
Chinese Clinical Oncology Jul 2017Meningiomas are frequent intracranial and intraspinal tumors. They are tumors of the elderly, and meningioma growth at certain localizations, as well as recurrent tumors... (Review)
Review
Meningiomas are frequent intracranial and intraspinal tumors. They are tumors of the elderly, and meningioma growth at certain localizations, as well as recurrent tumors or primary aggressive biology may pose a therapeutic challenge. To understand the growth characteristics of meningiomas, animal models can provide insights both from a biological and therapeutical point of view. Using genetically-engineered mouse models (GEMM), it has been proven that alterations of the neurofibromatosis type 2 (NF2) gene are key steps for benign meningioma development. Aggressive meningiomas can be induced by simultaneous activation of Nf2 and the PDGF (platelet-derived growth factor)/-PDGF-Receptor (R) system, or inactivation of Tp53 and cdkn2ab in mice. However, mechanisms acting in NF2 wild-type meningiomas are poorly understood so far, because appropriate models are lacking. Xenograft models have been used either by implantation of primary cultures derived from human meningiomas, or immortalized human cell lines, respectively. While the value of primary cells is limited due to low rate of overall tumor growth and slow proliferation, xenograft approaches have been shown to be helpful for the evaluation of potential medical treatment options. Future studies must incorporate new molecular meningioma tumor drivers, as well as potential treatment options based on recurrent genetic alterations into the generation of meningioma models.
Topics: Animals; Disease Models, Animal; Genes, Neurofibromatosis 2; Heterografts; Humans; Meningeal Neoplasms; Meningioma; Mice; Neoplasm Recurrence, Local
PubMed: 28595424
DOI: 10.21037/cco.2017.05.03 -
Handbook of Clinical Neurology 2020Information on the vascular supply to meningiomas is critical to the neurosurgeon. Most meningiomas are supplied by the external carotid artery, though many get pial...
Information on the vascular supply to meningiomas is critical to the neurosurgeon. Most meningiomas are supplied by the external carotid artery, though many get pial contribution as well. Angiogenesis is critical for these neoplasms to grow. Vascular endothelial growth factor (VEGF) has been a popular target of research to decrease angiogenesis. Peritumoral brain edema (PTBE) is occasionally seen in meningiomas, which makes surgical resection more challenging. The exact cause of PTBE remains unclear, but a number of factors have been postulated to contribute. Assessment of the vascularity of meningiomas is best carried out with angiography, but noninvasive techniques are improving, diminishing the need for more invasive imaging. Embolization of tumors can be performed to minimize perioperative blood loss and potentially lower surgical morbidity. However, it has not been shown to improve outcomes, and procedural risks exist. Higher grade tumors commonly have higher vascularity. Higher vascular meningiomas are more likely to recur and have higher levels of VEGF. The vascularity of meningiomas remains a topic of interest and is the focus of many research projects.
Topics: Aged; Brain Edema; Female; Humans; Male; Meningeal Neoplasms; Meningioma; Middle Aged; Neoplasm Recurrence, Local; Vascular Endothelial Growth Factor A
PubMed: 32553286
DOI: 10.1016/B978-0-12-804280-9.00010-X -
Current Treatment Options in Oncology May 2019Recurrent and refractory meningiomas are a clinical challenge and treatment at the time of recurrence is not well delineated. Treatment with surgery and/or radiation... (Review)
Review
Recurrent and refractory meningiomas are a clinical challenge and treatment at the time of recurrence is not well delineated. Treatment with surgery and/or radiation remain the mainstay, but each has their limitations and risks. The search for an adjuvant systemic therapy continues and as many of the initially promising approaches have not had reproducible responses. Bevacizumab has shown some efficacy in controlling recurrent disease and could be useful in disease that is multifocal or in close proximity to critical structures. Other targeted therapies, as well as immunotherapy, are being studied and trials are in development. Though we are hopeful that these novel therapies will benefit patients with refractory meningiomas, we approach them with some trepidation. This is due to prior failures of immunotherapy and targeted therapy in central nervous system disease. In addition, there is known difficulty in developing trials and assessing response with these slow-growing tumors.
Topics: Combined Modality Therapy; Disease Management; Drug Resistance, Neoplasm; Humans; Meningeal Neoplasms; Meningioma; Recurrence; Retreatment; Treatment Outcome
PubMed: 31073797
DOI: 10.1007/s11864-019-0648-z -
Handbook of Clinical Neurology 2020We know that the extent of resection is the greatest predictor of long-term survival. However, the potential tradeoff for gross total resection in difficult locations is... (Review)
Review
We know that the extent of resection is the greatest predictor of long-term survival. However, the potential tradeoff for gross total resection in difficult locations is diminished quality of life. Benign subtotally resected or small incidentally discovered meningiomas may be followed clinically especially in the elderly. In addition, radiosurgery plays a role in the treatment of meningiomas as a primary treatment modality, or as a salvage therapy. Decisions regarding management should be made with an understanding of the natural history and rate of growth. In this chapter we review the known meningioma epidemiology as well as the growth patterns of meningiomas based upon location.
Topics: Humans; Meningeal Neoplasms; Meningioma; Microsurgery; Neoplasm Recurrence, Local; Quality of Life
PubMed: 32553291
DOI: 10.1016/B978-0-12-804280-9.00014-7 -
Journal of Cutaneous Pathology Feb 2023Cutaneous meningiomas are rare tumors of ectopic meningeal tissue in the dermis or subcutis and are most commonly located on the scalp and around the eyes, ears, nose,...
Cutaneous meningiomas are rare tumors of ectopic meningeal tissue in the dermis or subcutis and are most commonly located on the scalp and around the eyes, ears, nose, and mouth. We present a case of cutaneous meningioma with an unusual presentation on the upper arm, but with positivity for epithelial membrane antigen to support the diagnosis of meningioma. Lack of pancytokeratin AE1/3 and p63 allowed us to rule out a keratinocytic tumor, such as squamous cell carcinoma and basal cell carcinoma. The absence of smooth muscle actin, FXIIIa, CD163, and CD34 allowed us to rule out a leiomyoma, dermatofibroma, and vascular neoplasm respectively. Weak staining of S100 allowed us to rule out a perineuroma. The purpose of this case report is to increase awareness of a rare presentation of cutaneous meningioma not located within the scalp/face region. This may broaden its inclusion within differential diagnoses of neoplasms of uncertain behavior and encourage correct clinical diagnosis, thus improving treatment outcomes.
Topics: Humans; Meningioma; Skin Neoplasms; Carcinoma, Squamous Cell; Treatment Outcome; Diagnosis, Differential; Meningeal Neoplasms
PubMed: 35996326
DOI: 10.1111/cup.14316